amyloidosis

Summary

Summary: A group of sporadic, familial and/or inherited, degenerative, and infectious disease processes, linked by the common theme of abnormal protein folding and deposition of AMYLOID. As the amyloid deposits enlarge they displace normal tissue structures, causing disruption of function. Various signs and symptoms depend on the location and size of the deposits.

Top Publications

  1. ncbi Amyloid fibril protein nomenclature: 2012 recommendations from the Nomenclature Committee of the International Society of Amyloidosis
    Jean D Sipe
    Department of Biochemistry, Boston University School of Medicine, MA, USA
    Amyloid 19:167-70. 2012
  2. ncbi Early-onset amyloid deposition and cognitive deficits in transgenic mice expressing a double mutant form of amyloid precursor protein 695
    M A Chishti
    Centre for Research in Neurodegenerative Diseases, The Department of Laboratory Medicine, Division of Neurology, University Health Network, University of Toronto, Toronto, Ontario M5S 3H2, Canada
    J Biol Chem 276:21562-70. 2001
  3. ncbi Noncontrast T1 mapping for the diagnosis of cardiac amyloidosis
    Theodoros D Karamitsos
    University of Oxford Centre for Clinical Magnetic Resonance Research, Department of Cardiovascular Medicine, John Radcliffe Hospital, Oxford, United Kingdom
    JACC Cardiovasc Imaging 6:488-97. 2013
  4. ncbi Molecular mechanisms of amyloidosis
    Giampaolo Merlini
    Amyloid Center, Biotechnology Research Laboratory, University Hospital IRCCS Policlinico San Matteo, Pavia, Italy
    N Engl J Med 349:583-96. 2003
  5. pmc Outcome of AL amyloidosis after high-dose melphalan and autologous stem cell transplantation: long-term results in a series of 421 patients
    Maria Teresa Cibeira
    Amyloid Treatment and Research Program, Department of Medicine, Boston University School of Medicine, Boston, MA 02118, USA
    Blood 118:4346-52. 2011
  6. pmc Amyloid ion channels: a common structural link for protein-misfolding disease
    Arjan Quist
    Neuroscience Research Institute, University of California, Santa Barbara, CA 93106, USA
    Proc Natl Acad Sci U S A 102:10427-32. 2005
  7. pmc Conformational conversion during amyloid formation at atomic resolution
    Timo Eichner
    Astbury Centre for Structural Molecular Biology and Institute of Molecular and Cellular Biology, University of Leeds, Leeds LS2 9JT, UK
    Mol Cell 41:161-72. 2011
  8. ncbi Prediction of "aggregation-prone" and "aggregation-susceptible" regions in proteins associated with neurodegenerative diseases
    Amol P Pawar
    Department of Chemistry, University of Cambridge, Lensfield Road, Cambridge CB2 1EW, UK
    J Mol Biol 350:379-92. 2005
  9. ncbi Green tea epigallocatechin-3-gallate (EGCG) modulates amyloid precursor protein cleavage and reduces cerebral amyloidosis in Alzheimer transgenic mice
    Kavon Rezai-Zadeh
    Silver Child Development Center, Department of Psychiatry and Behavioral Medicine, University of South Florida, Tampa, Florida 33613, USA
    J Neurosci 25:8807-14. 2005
  10. pmc Tissue damage in the amyloidoses: Transthyretin monomers and nonnative oligomers are the major cytotoxic species in tissue culture
    Natalia Reixach
    Division of Rheumatology Research, Department of Molecular and Experimental Medicine, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, CA 92037, USA
    Proc Natl Acad Sci U S A 101:2817-22. 2004

Research Grants

  1. Reactive (AA)Amyloidosis
    Merrill D Benson; Fiscal Year: 2013
  2. Vascular dysfunction and oxidative stress in primary amyloidosis
    RAYMOND QUEZON MIGRINO; Fiscal Year: 2010
  3. Systemic AA Amyloidosis Inhibitors
    ALAN D SNOW; Fiscal Year: 2010
  4. Beta Cell Adaptation to Stress in Baboon Pancreas After Partial Pancreatectomy
    FRANCO BATTISTA FOLLI; Fiscal Year: 2010
  5. Improving CBF through direct control of CSF pulsations
    MARK GREGORY LUCIANO; Fiscal Year: 2010
  6. Regulation of HMGB1 Release in Endotoxemia
    Haichao Wang; Fiscal Year: 2013
  7. Nanoliposome-based Treatment of Amyloid Protein (AL) Toxicity
    RAYMOND QUEZON MIGRINO; Fiscal Year: 2013
  8. Molecular Mechanisms and Treatment of Primary Amyloid AL Cardiomyopathy
    Ronglih Liao; Fiscal Year: 2013
  9. The Abeta protein of Alzheimer's Disease is an antimicrobial peptide
    Robert D Moir; Fiscal Year: 2013
  10. MEMBRANE FUSION IN RETINAL ROD OUTER SEGMENTS
    KATHLEEN BOESZEBATTAGLIA; Fiscal Year: 2012

Detail Information

Publications399 found, 100 shown here

  1. ncbi Amyloid fibril protein nomenclature: 2012 recommendations from the Nomenclature Committee of the International Society of Amyloidosis
    Jean D Sipe
    Department of Biochemistry, Boston University School of Medicine, MA, USA
    Amyloid 19:167-70. 2012
    The Nomenclature Committee of the International Society of Amyloidosis (ISA) met during the XIIIth International Symposium, May 6-10, 2012, Groningen, The Netherlands, to formulate recommendations on amyloid fibril protein nomenclature ..
  2. ncbi Early-onset amyloid deposition and cognitive deficits in transgenic mice expressing a double mutant form of amyloid precursor protein 695
    M A Chishti
    Centre for Research in Neurodegenerative Diseases, The Department of Laboratory Medicine, Division of Neurology, University Health Network, University of Toronto, Toronto, Ontario M5S 3H2, Canada
    J Biol Chem 276:21562-70. 2001
    ..The Tg mice described here suggest a potential to investigate aspects of Alzheimer's disease pathogenesis, prophylaxis, and therapy within short time frames...
  3. ncbi Noncontrast T1 mapping for the diagnosis of cardiac amyloidosis
    Theodoros D Karamitsos
    University of Oxford Centre for Clinical Magnetic Resonance Research, Department of Cardiovascular Medicine, John Radcliffe Hospital, Oxford, United Kingdom
    JACC Cardiovasc Imaging 6:488-97. 2013
    This study sought to explore the potential role of noncontrast myocardial T1 mapping for detection of cardiac involvement in patients with primary amyloid light-chain (AL) amyloidosis.
  4. ncbi Molecular mechanisms of amyloidosis
    Giampaolo Merlini
    Amyloid Center, Biotechnology Research Laboratory, University Hospital IRCCS Policlinico San Matteo, Pavia, Italy
    N Engl J Med 349:583-96. 2003
  5. pmc Outcome of AL amyloidosis after high-dose melphalan and autologous stem cell transplantation: long-term results in a series of 421 patients
    Maria Teresa Cibeira
    Amyloid Treatment and Research Program, Department of Medicine, Boston University School of Medicine, Boston, MA 02118, USA
    Blood 118:4346-52. 2011
    Previous studies have suggested that, in patients with AL amyloidosis treated with high-dose melphalan and autologous stem-cell transplantation (HDM/SCT), the greatest benefit is seen in those patients achieving a hematologic complete ..
  6. pmc Amyloid ion channels: a common structural link for protein-misfolding disease
    Arjan Quist
    Neuroscience Research Institute, University of California, Santa Barbara, CA 93106, USA
    Proc Natl Acad Sci U S A 102:10427-32. 2005
    ..These ion channels would destabilize cellular ionic homeostasis and hence induce cell pathophysiology and degeneration in amyloid diseases...
  7. pmc Conformational conversion during amyloid formation at atomic resolution
    Timo Eichner
    Astbury Centre for Structural Molecular Biology and Institute of Molecular and Cellular Biology, University of Leeds, Leeds LS2 9JT, UK
    Mol Cell 41:161-72. 2011
    ....
  8. ncbi Prediction of "aggregation-prone" and "aggregation-susceptible" regions in proteins associated with neurodegenerative diseases
    Amol P Pawar
    Department of Chemistry, University of Cambridge, Lensfield Road, Cambridge CB2 1EW, UK
    J Mol Biol 350:379-92. 2005
    ....
  9. ncbi Green tea epigallocatechin-3-gallate (EGCG) modulates amyloid precursor protein cleavage and reduces cerebral amyloidosis in Alzheimer transgenic mice
    Kavon Rezai-Zadeh
    Silver Child Development Center, Department of Psychiatry and Behavioral Medicine, University of South Florida, Tampa, Florida 33613, USA
    J Neurosci 25:8807-14. 2005
    ..These data raise the possibility that EGCG dietary supplementation may provide effective prophylaxis for AD...
  10. pmc Tissue damage in the amyloidoses: Transthyretin monomers and nonnative oligomers are the major cytotoxic species in tissue culture
    Natalia Reixach
    Division of Rheumatology Research, Department of Molecular and Experimental Medicine, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, CA 92037, USA
    Proc Natl Acad Sci U S A 101:2817-22. 2004
    ..The studies are consistent with a model in which the misfolded TTR monomer rapidly aggregates to form transient low molecular mass assemblies (<100 kDa) that are highly cytotoxic in tissue culture...
  11. pmc Non-invasive evaluation of the myocardial substrate of cardiac amyloidosis by gadolinium cardiac magnetic resonance
    E Perugini
    Institute of Cardiology, University of Bologna, S Orsola Malpighi Hospital, Bologna, Italy
    Heart 92:343-9. 2006
    ..the prevalence and distribution of gadolinium (Gd) enhancement at cardiac magnetic resonance (CMR) imaging in patients with cardiac amyloidosis (CA) and to look for associations with clinical, morphological, and functional features.
  12. ncbi Amyloidosis in autoinflammatory syndromes
    Laura Obici
    Amyloidosis Research and Treatment Centre, Biotechnology Research Laboratories, Fondazione IRCCS Policlinico San Matteo, Italy
    Autoimmun Rev 12:14-7. 2012
    AA amyloidosis may still dramatically impact on the outcome of patients with autoinflammatory diseases, particularly when diagnosis is delayed...
  13. pmc Low-density lipoprotein receptor overexpression enhances the rate of brain-to-blood Aβ clearance in a mouse model of β-amyloidosis
    Joseph M Castellano
    Department of Neurology, Hope Center for Neurological Disorders, Charles F and Joanne Knight Alzheimer s Disease Research Center, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 109:15502-7. 2012
    ..Together, our results suggest a unique mechanism by which LDLR regulates brain-to-blood Aβ clearance, which may serve as a useful therapeutic avenue in targeting Aβ clearance from the brain...
  14. pmc Anti-apoE immunotherapy inhibits amyloid accumulation in a transgenic mouse model of Aβ amyloidosis
    Jungsu Kim
    Department of Neurology, Washington University School of Medicine, Saint Louis, MO 63110, USA
    J Exp Med 209:2149-56. 2012
    ..Our data also have important broader implications for other amyloidosis. Immunotherapy to proteins tightly associated with misfolded proteins might open up a new treatment option for ..
  15. pmc Induction of cerebral beta-amyloidosis: intracerebral versus systemic Abeta inoculation
    Yvonne S Eisele
    Department of Cellular Neurology, Hertie Institute for Clinical Brain Research, University of Tubingen, D 72076 Tubingen, Germany
    Proc Natl Acad Sci U S A 106:12926-31. 2009
    ..Here we show that induction of cerebral beta-amyloidosis can be achieved in many different brain areas of APP23 transgenic mice through the injection of dilute Abeta-..
  16. ncbi Diet-induced insulin resistance promotes amyloidosis in a transgenic mouse model of Alzheimer's disease
    Lap Ho
    Neuroinflammation Research Laboratories, Department of Psychiatry, Mount Sinai School of Medicine, New York, New York, USA
    FASEB J 18:902-4. 2004
    ..In this study we examined the role of dietary conditions leading to NIDDM-like insulin resistance on amyloidosis in Tg2576 mice, which model AD-like neuropathology...
  17. ncbi Amyloid fibril protein nomenclature: 2010 recommendations from the nomenclature committee of the International Society of Amyloidosis
    Jean D Sipe
    Department of Biochemistry Emerita, Boston University School of Medicine, Boston, MA 02118, USA
    Amyloid 17:101-4. 2010
    ..Current nomenclature lists of 27 human and nine animal fibril proteins are provided together with a list of eight inclusion bodies that exhibit some of the properties of amyloid fibrils...
  18. ncbi Intake of sucrose-sweetened water induces insulin resistance and exacerbates memory deficits and amyloidosis in a transgenic mouse model of Alzheimer disease
    Dongfeng Cao
    Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama 35294, USA
    J Biol Chem 282:36275-82. 2007
    ..that the up-regulation of apoE accelerated the aggregation of Abeta, resulting in the exacerbation of cerebral amyloidosis in sucrose-treated mice...
  19. ncbi Receptor-dependent cell stress and amyloid accumulation in systemic amyloidosis
    S D Yan
    Departments of Pathology, Physiology, and Surgery, College of Physicians and Surgeons of Columbia University, New York, New York 10032, USA
    Nat Med 6:643-51. 2000
    ..Antagonizing RAGE suppressed cell stress and amyloid deposition in mouse spleens. These data indicate that RAGE is a potential target for inhibiting accumulation of amyloid A and for limiting cellular dysfunction induced by amyloid A...
  20. ncbi Infusion of light chains from patients with cardiac amyloidosis causes diastolic dysfunction in isolated mouse hearts
    R Liao
    Cardiac Muscle Research Laboratory, Amyloid Treatment and Research Program, Department of Medicine, Boston University School of Medicine, Boston, Massachusetts, USA
    Circulation 104:1594-7. 2001
    Primary (AL) amyloidosis is a plasma cell dyscrasia characterized by clonal production of immunoglobulin light chains (LC) resulting in the subsequent systemic deposition of extracellular amyloid fibrils...
  21. pmc A{beta} accelerates the spatiotemporal progression of tau pathology and augments tau amyloidosis in an Alzheimer mouse model
    David E Hurtado
    Department of Pathology, Laboratory Medicine, Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 4283, USA
    Am J Pathol 177:1977-88. 2010
    ..More significantly, the presence of Aβ accelerated NFT formation and enhanced tau amyloidosis; however, tau pathology did not have the same effect on Aβ pathology...
  22. ncbi Telocytes in human isolated atrial amyloidosis: ultrastructural remodelling
    E Mandache
    Victor Babes National Institute of Pathology, Bucharest, Romania
    J Cell Mol Med 14:2739-47. 2010
    The human heart can be frequently affected by an organ-limited amyloidosis called isolated atrial amyloidosis (IAA). IAA is a frequent histopathological finding in patients with long-standing atrial fibrillation (AF)...
  23. pmc Laser microdissection and mass spectrometry-based proteomics aids the diagnosis and typing of renal amyloidosis
    Sanjeev Sethi
    Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA
    Kidney Int 82:226-34. 2012
    Accurate diagnosis and typing of renal amyloidosis is critical for prognosis, genetic counseling, and treatment. Laser microdissection and mass spectrometry are emerging techniques for the analysis and diagnosis of many renal diseases...
  24. ncbi Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis
    Belinda Ng
    Amyloidosis Treatment and Research Program, Sections of Cardiology, Boston University School of Medicine, Boston, MA, USA
    Arch Intern Med 165:1425-9. 2005
    ..However, extensive deposition of transthyretin-derived amyloid fibrils in the heart (senile systemic amyloidosis [SSA]) can cause heart failure...
  25. ncbi Role of cardiac magnetic resonance imaging in the detection of cardiac amyloidosis
    Imran S Syed
    Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota 55905, USA
    JACC Cardiovasc Imaging 3:155-64. 2010
    ..role and mechanism of late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) in identifying cardiac amyloidosis (CA) and to investigate associations between LGE and clinical, morphologic, functional, and biochemical features...
  26. ncbi Diagnostic tools for amyloidosis
    Eric Hachulla
    Internal Medicine Department, Centre Hospitalier Regional et Universitaire, Hopital Claude Huriez, 59037, Lille Cedex, France
    Joint Bone Spine 69:538-45. 2002
    Demonstration of amyloid deposits in biopsy specimens is the only means of confirming the diagnosis of amyloidosis. In experienced hands, nonsurgical biopsies of the rectal mucosa or, preferably, of the abdominal fat pad or labial ..
  27. pmc Magic angle spinning NMR analysis of beta2-microglobulin amyloid fibrils in two distinct morphologies
    Galia T Debelouchina
    Department of Chemistry and Francis Bitter Magnet Laboratory, Massachusetts Institute of Technology, Cambridge, Massachusetts 02139, USA
    J Am Chem Soc 132:10414-23. 2010
    ..is the major structural component of amyloid fibrils deposited in a condition known as dialysis-related amyloidosis. Despite numerous studies that have elucidated important aspects of the fibril formation process in vitro, and ..
  28. ncbi Increased lipid peroxidation precedes amyloid plaque formation in an animal model of Alzheimer amyloidosis
    D Pratico
    Center for Experimental Therapeutics and Department of Pharmacology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Neurosci 21:4183-7. 2001
    ..in vivo is associated with increased LPO, we examined iP levels in a transgenic mouse model (Tg2576) of AD amyloidosis. Urine, plasma, and brain tissues were collected from Tg2576 and littermate wild-type (WT) animals at different ..
  29. ncbi Atrial amyloidosis: an arrhythmogenic substrate for persistent atrial fibrillation
    Christoph Rocken
    Department of Pathology, Otto von Guericke University, Magdeburg, Germany
    Circulation 106:2091-7. 2002
    Structural changes, like atrial fibrosis, may increase the likelihood of atrial fibrillation (AF) occurring in response to triggering events. The influence of isolated atrial amyloidosis (IAA) is largely unknown.
  30. ncbi Weekly and twice-weekly bortezomib in patients with systemic AL amyloidosis: results of a phase 1 dose-escalation study
    Donna E Reece
    Princess Margaret Hospital, University Health Network, Toronto, ON, Canada
    Blood 114:1489-97. 2009
    New treatment options are required for primary systemic AL amyloidosis (AL). This phase 1 dose-escalation component of a phase 1/2 study in relapsed AL aimed to determine the maximum tolerated dose (MTD) of bortezomib once weekly (0.7-1...
  31. ncbi Human lysozyme gene mutations cause hereditary systemic amyloidosis
    M B Pepys
    Department of Medicine, Royal Postgraduate Medical School, Hammersmith Hospital, London, UK
    Nature 362:553-7. 1993
    Hereditary non-neuropathic systemic amyloidosis (Ostertag-type) is a rare autosomal dominant disease in which amyloid deposition in the viscera is usually fatal by the fifth decade...
  32. ncbi Abeta is targeted to the vasculature in a mouse model of hereditary cerebral hemorrhage with amyloidosis
    Martin C Herzig
    Department of Cellular Neurology, Hertie Institute for Clinical Brain Research, University of Tubingen, D 72076 Tubingen, Germany
    Nat Neurosci 7:954-60. 2004
    ..In contrast to Alzheimer disease (AD), the brains of those affected by hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D) show few parenchymal amyloid plaques...
  33. ncbi Cardiovascular magnetic resonance in cardiac amyloidosis
    Alicia Maria Maceira
    Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK
    Circulation 111:186-93. 2005
    Cardiac amyloidosis can be diagnostically challenging. Cardiovascular magnetic resonance (CMR) can assess abnormal myocardial interstitium.
  34. pmc Haploinsufficiency of human APOE reduces amyloid deposition in a mouse model of amyloid-β amyloidosis
    Jungsu Kim
    Department of Neurology, Developmental Biology, Hope Center for Neurological Disorders, Knight Alzheimer s Disease Research Center, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Neurosci 31:18007-12. 2011
    ..Like in humans, apoE4 expression in animal models that develop Aβ amyloidosis results in greater Aβ and amyloid deposition than with apoE3 expression...
  35. ncbi Alzheimer's-type amyloidosis in transgenic mice impairs survival of newborn neurons derived from adult hippocampal neurogenesis
    Laure Verret
    Centre National de la Recherche Scientifique, Universite Paul Sabatier, 31062 Toulouse, France
    J Neurosci 27:6771-80. 2007
    ..Diminished capacity for hippocampal neuron replacement may contribute to the cognitive decline observed in these mice...
  36. ncbi GM-CSF upregulated in rheumatoid arthritis reverses cognitive impairment and amyloidosis in Alzheimer mice
    Tim D Boyd
    USF Health Byrd Alzheimer s Center and Research Institute, Tampa, FL 33613, USA
    J Alzheimers Dis 21:507-18. 2010
    ..G-CSF and more significantly, GM-CSF reduced amyloidosis throughout the treated brain hemisphere one week following bolus administration to AD mice...
  37. ncbi Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, 18-22 April 2004
    Morie A Gertz
    Dysproteinemia Clinic, Mayo Clinic, Rochester, Minnesota 55905, USA
    Am J Hematol 79:319-28. 2005
    ..definition of organ involvement and response for patients on treatment protocols for immunoglobulin light-chain amyloidosis (AL)...
  38. ncbi Natural history and outcome in systemic AA amyloidosis
    Helen J Lachmann
    National Amyloidosis Centre and Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine, Royal Free and University College Medical School, London
    N Engl J Med 356:2361-71. 2007
    ..amyloid fibrils derived from circulating acute-phase reactant serum amyloid A protein (SAA) causes systemic AA amyloidosis, a serious complication of many chronic inflammatory disorders...
  39. pmc Antibodies to human serum amyloid P component eliminate visceral amyloid deposits
    Karl Bodin
    Centre for Amyloidosis and Acute Phase Proteins, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK
    Nature 468:93-7. 2010
    Accumulation of amyloid fibrils in the viscera and connective tissues causes systemic amyloidosis, which is responsible for about one in a thousand deaths in developed countries...
  40. ncbi Vitamin E reduces amyloidosis and improves cognitive function in Tg2576 mice following repetitive concussive brain injury
    Valeria Conte
    Head Injury Center, University of Pennsylvania, Philadelphia, Pennsylvania, USA
    J Neurochem 90:758-64. 2004
    ..This study suggests that the exacerbation of brain oxidative stress following RCBI plays a mechanistic role in accelerating Alphabeta accumulation and behavioral impairments in the Tg2576 mice...
  41. ncbi Amyloidosis and Alzheimer's disease
    Jorge Ghiso
    Department of Pathology and Psychiatry, New York University School of Medicine, New York, NY 10016, USA
    Adv Drug Deliv Rev 54:1539-51. 2002
    Alzheimer's disease (AD) is the most frequent type of amyloidosis in humans and the commonest form of dementia...
  42. pmc Associations between serum cholesterol levels and cerebral amyloidosis
    Bruce Reed
    University of California, Davis
    JAMA Neurol 71:195-200. 2014
    ..Whether elevated serum cholesterol levels act as such a factor is unknown...
  43. pmc Mysterious oligomerization of the amyloidogenic proteins
    Vladimir N Uversky
    Center for Computational Biology and Bioinformatics, Indiana University School of Medicine, Indianapolis, IN 46202, USA
    FEBS J 277:2940-53. 2010
    ..This review provides an overview of some topics related to soluble oligomers and represents several illustrative examples of toxic, nontoxic, productive and off-pathway amyloid oligomers...
  44. pmc Transmissibility of systemic amyloidosis by a prion-like mechanism
    Katarzyna Lundmark
    Division of Molecular and Immunological Pathology, Linkoping University, 581 83 Linkoping, Sweden
    Proc Natl Acad Sci U S A 99:6979-84. 2002
    ..This phenomenon is evidenced in vivo by the fact that amyloid protein A (AA) amyloidosis in mice is markedly accelerated when the animals are given, in addition to an inflammatory stimulus, an i.v...
  45. pmc Heterogeneity in primary structure, post-translational modifications, and germline gene usage of nine full-length amyloidogenic kappa1 immunoglobulin light chains
    Lawreen H Connors
    Alan and Sandra Gerry Amyloid Research Laboratory in the Amyloid Treatment and Research Program, Boston University School of Medicine, Boston, Massachusetts 02118, USA
    Biochemistry 46:14259-71. 2007
    Immunoglobulin light chain amyloidosis is a protein misfolding disease in which a monoclonal immunoglobulin (Ig) light chain (LC) with a critically folded beta-conformation self-aggregates to form highly ordered, nonbranching amyloid ..
  46. pmc Mice with alopecia, osteoporosis, and systemic amyloidosis due to mutation in Zdhhc13, a gene coding for palmitoyl acyltransferase
    Amir N Saleem
    Institute of Biomedical Sciences, Academia Sinica, Nankang, Taiwan
    PLoS Genet 6:e1000985. 2010
    ..thrive, shortened life span, skin and hair abnormalities including alopecia, severe osteoporosis, and systemic amyloidosis (both AA and AL amyloids depositions)...
  47. ncbi Partially folded intermediates as critical precursors of light chain amyloid fibrils and amorphous aggregates
    R Khurana
    Department of Chemistry and Biochemistry, University of California, Santa Cruz 95064, USA
    Biochemistry 40:3525-35. 2001
    Light chain, or AL, amyloidosis is a pathological condition arising from systemic extracellular deposition of monoclonal immunoglobulin light chain variable domains in the form of insoluble amyloid fibrils, especially in the kidneys...
  48. ncbi Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types
    Claudio Rapezzi
    Istituto di Cardiologia, Policlinico S Orsola Malpighi, Via Massarenti n 9, 40125 Bologna, Italy
    Circulation 120:1203-12. 2009
    ..In this study, we compared the diagnostic/clinical profiles of these 3 types of systemic cardiac amyloidosis.
  49. ncbi Beta-sheet breaker peptides inhibit fibrillogenesis in a rat brain model of amyloidosis: implications for Alzheimer's therapy
    C Soto
    Department of Pathology, New York University Medical Center, New York 10016, USA
    Nat Med 4:822-6. 1998
    ..beta-protein deposition in vivo and completely blocks the formation of amyloid fibrils in a rat brain model of amyloidosis. These findings may provide the basis for a new therapeutic approach to prevent amyloidosis in Alzheimer's ..
  50. ncbi Therapeutic actions of insulin-like growth factor I on APP/PS2 mice with severe brain amyloidosis
    E Carro
    Laboratory of Neuroendocrinology, Cajal Institute, CSIC, Avda Dr Arce 37, 28002 Madrid Spain
    Neurobiol Aging 27:1250-7. 2006
    ..mutant forms of both amyloid-beta (Abeta) precursor protein (APP) and presenilin (PS) 2 develop severe brain amyloidosis and cognitive deficits, two pathological hallmarks of Alzheimer's disease (AD)...
  51. ncbi Brain deposition of beta-amyloid is a common pathologic feature in HIV positive patients
    Douglas A Green
    Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA
    AIDS 19:407-11. 2005
    ..Our working hypothesis is that long term survival, aging and the secondary effects of HAART may contribute to increased beta-amyloid accumulation in this patient population...
  52. ncbi Immunoglobulin light chain amyloidosis: 2013 update on diagnosis, prognosis, and treatment
    Morie A Gertz
    Division of Hematology, Mayo Clinic, Rochester, MN 55905, USA
    Am J Hematol 88:416-25. 2013
    Immunoglobulin (Ig) light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of Ig light chain are deposited in tissues...
  53. ncbi Oral administration of histone deacetylase inhibitor MS-275 ameliorates neuroinflammation and cerebral amyloidosis and improves behavior in a mouse model
    Zhi Yuan Zhang
    Division of Immunopathology of the Nervous System, Institute of Pathology and Neuropathology, University of Tubingen, Tubingen, Germany
    J Neuropathol Exp Neurol 72:178-85. 2013
    ..inhibitor, MS-275, was orally administered by gavage to transgenic APP/PS1 mice, an animal model of cerebral amyloidosis for Alzheimer disease...
  54. ncbi Ptosis from localized A-lambda-amyloid deposits in the levator palpebrae muscle
    Stefan Dithmar
    Department of Ophthalmology, University of Heidelberg, Heidelberg, Germany
    Ophthalmology 111:1043-7. 2004
    ..To describe a patient with unilateral ptosis caused by localized amyloid deposits of immunoglobulin lambda-light chains (A-lambda-amyloid) in the levator palpebrae muscle...
  55. ncbi Outcome of autologous stem cell transplantation for AL amyloidosis in the UK
    Hugh J B Goodman
    National Amyloidosis Centre, Centre for Amyloidosis and Acute Phase Proteins, Royal Free and University College Medical Schools, Hampstead Campus, Royal Free Hospital, London, UK
    Br J Haematol 134:417-25. 2006
    ..chemotherapy with autologous stem cell transplantation (SCT) is widely used as a treatment for systemic AL amyloidosis, but its efficacy has not been proved and it has substantial toxicity in this setting...
  56. ncbi Noninvasive diagnosis of biopsy-proven cardiac amyloidosis
    Joseph E Rahman
    Division of Cardiology, Department of Medicine, Johns Hopkins Bayview Medical Center, 4940 Eastern Avenue, Baltimore, MD 21224 2780, USA
    J Am Coll Cardiol 43:410-5. 2004
    This study analyzed the utility of electrocardiographic (ECG) and echocardiographic findings in the diagnosis of amyloidosis proven by endomyocardial biopsy.
  57. ncbi A novel lysozyme mutation Phe57Ile associated with hereditary renal amyloidosis
    Masahide Yazaki
    Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana 46202, USA
    Kidney Int 63:1652-7. 2003
    ..forms of lysozyme, a ubiquitous bacteriolytic enzyme, are known to lead to hereditary non-neuropathic renal amyloidosis and, so far, three different mutations of the lysozyme gene have been reported...
  58. pmc Loss of LR11/SORLA enhances early pathology in a mouse model of amyloidosis: evidence for a proximal role in Alzheimer's disease
    Sara E Dodson
    Center for Neurodegenerative Disease, Emory University, Atlanta, Georgia 30322, USA
    J Neurosci 28:12877-86. 2008
    ..APP processing and Abeta accumulation in vivo and is of proximal importance to the cascade of pathological amyloidosis. The results of the current study support the hypothesis that control of LR11 expression may exert critical ..
  59. ncbi Amyloid heart disease
    Rodney H Falk
    Harvard Vanguard Medical Associates, Harvard Medical School, Boston, MA 02215, USA
    Prog Cardiovasc Dis 52:347-61. 2010
    ..Cardiac deposition, leading to an infiltrative/restrictive cardiomyopathy, is a common feature of amyloidosis. It may be the presenting feature of the disease or may be discovered while investigating a patient presenting ..
  60. ncbi Bortezomib with or without dexamethasone in primary systemic (light chain) amyloidosis
    Efstathios Kastritis
    Department of Clinical Therapeutics, University of Athens School of Medicine, 80 Vas Sofias Ave, 115 28, Athens, Greece
    J Clin Oncol 28:1031-7. 2010
    ..or without dexamethasone and to define prognostic factors for patients with primary systemic light chain (AL) amyloidosis treated with bortezomib or both...
  61. ncbi Noninvasive etiologic diagnosis of cardiac amyloidosis using 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy
    Enrica Perugini
    Institute of Cardiology, University of Bologna and S Orsola Malpighi Hospital, Bologna, Italy
    J Am Coll Cardiol 46:1076-84. 2005
    We investigated the diagnostic accuracy of 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) scintigraphy for differentiation of monoclonal immunoglobulin light-chain (AL) and transthyretin (TTR)-related cardiac amyloidosis.
  62. pmc The transthyretin amyloidoses: from delineating the molecular mechanism of aggregation linked to pathology to a regulatory-agency-approved drug
    Steven M Johnson
    Department of Chemistry and The Skaggs Institute for Chemical Biology, The Scripps Research Institute, La Jolla, CA 92037, USA
    J Mol Biol 421:185-203. 2012
    ..Here we review the biophysical basis for the kinetic stabilization strategy and the structure-based drug design effort that led to this first-in-class pharmacologic agent...
  63. ncbi Calorie restriction attenuates Alzheimer's disease type brain amyloidosis in Squirrel monkeys (Saimiri sciureus)
    Weiping Qin
    Department of Psychiatry, Mount Sinai School of Medicine, New York, NY 10029, USA
    J Alzheimers Dis 10:417-22. 2006
    ..In this study we continued to explore the role of CR in AD-type brain amyloidosis in Squirrel monkeys (Saimiri sciureus)...
  64. ncbi Monoclonal immunoglobulin deposition disease: light chain and light and heavy chain deposition diseases and their relation to light chain amyloidosis. Clinical features, immunopathology, and molecular analysis
    J N Buxbaum
    New York University School of Medicine, New York
    Ann Intern Med 112:455-64. 1990
    Monoclonal immunoglobulin deposition occurs in tissues as Congo Red binding fibrils in light chain amyloidosis, as less structured deposits in light chain deposition disease, and as similar but distinct deposits in light and heavy chain ..
  65. ncbi Hereditary renal amyloidosis caused by a new variant lysozyme W64R in a French family
    Sophie Valleix
    Laboratoire de Biochimie et Biologie Moleculaire, Hopital Cochin, Assistance Publique Hôpitaux de Paris AP HP et INSERM EMI 00 05, Paris, France
    Kidney Int 61:907-12. 2002
    The number of proteins with mutations resulting in amyloidosis has continued to increase...
  66. ncbi Successful tocilizumab treatment in a patient with adult-onset Still's disease complicated by chronic active hepatitis B and amyloid A amyloidosis
    Dai Kishida
    Department of Internal Medicine, Center for Rheumatic Diseases, Dohgo Spa Hospital, 21 21 Otsu Dohgo Himezuka, Matsuyama, Ehime, 790 0858, Japan
    Mod Rheumatol 21:215-8. 2011
    ..TCZ) use in a patient with adult-onset Still's disease (AOSD) complicated by chronic hepatitis B (CHB) and AA amyloidosis (AAA). Treatments with corticosteroid and various types of immunosuppressants were unsuccessful...
  67. ncbi Mechanisms of disease: monoclonal immunoglobulin deposition. Amyloidosis, light chain deposition disease, and light and heavy chain deposition disease
    J Buxbaum
    Medical Service, New York Department of Veterans Affairs Medical Center, New York
    Hematol Oncol Clin North Am 6:323-46. 1992
    ..Better understanding of the latter factors is necessary for the development of better modes of treatment...
  68. pmc Amyloidosis: pathogenesis and new therapeutic options
    Giampaolo Merlini
    Amyloidosis Research and Treatment Center, Foundation IRCCS Policlinico San Matteo, University of Pavia, P le Golgi, 19, 27100 Pavia, Italy
    J Clin Oncol 29:1924-33. 2011
    ..The most common type, immunoglobulin light chain amyloidosis (AL), is caused by clonal plasma cells that produce misfolded light chains...
  69. ncbi Nuclear imaging in cardiac amyloidosis
    A W J M Glaudemans
    Department of Nuclear Medicine and Molecular Imaging, University Medical Center Groningen, Groningen, The Netherlands
    Eur J Nucl Med Mol Imaging 36:702-14. 2009
    b>Amyloidosis is a disease characterized by depositions of amyloid in organs and tissues. It can be localized (in just one organ) or systemic...
  70. ncbi Human cystatin C, an amyloidogenic protein, dimerizes through three-dimensional domain swapping
    R Janowski
    Department of Crystallography, Faculty of Chemistry, A Mickiewicz University, Grunwaldzka 6, 60 780 Poznan, Poland
    Nat Struct Biol 8:316-20. 2001
    ..severe 'conformational disease' is associated with the L68Q mutant of human cystatin C, which causes massive amyloidosis, cerebral hemorrhage and death in young adults...
  71. ncbi Significance of SAA1.3 allele genotype in Japanese patients with amyloidosis secondary to rheumatoid arthritis
    T Nakamura
    Section of Internal Medicine and Rheumatology, Kumamoto Center for Arthritis and Rheumatology, 1 15 7 Kuhonji, Kumamoto 862 0976, Japan
    Rheumatology (Oxford) 45:43-9. 2006
    To clarify the clinical significance of the SAA1.3 allele in the development and outcome of AA amyloidosis in Japanese patients with rheumatoid arthritis (RA).
  72. ncbi Tocilizumab dramatically ameliorated life-threatening diarrhea due to secondary amyloidosis associated with rheumatoid arthritis
    Hiroe Sato
    Department of Rheumatology, Niigata Rheumatic Center, Shibata City, Niigata 957 0054, Japan
    Clin Rheumatol 28:1113-6. 2009
    ..of the humanized anti-interleukin-6 receptor antibody tocilizumab (TCZ) in a patient with secondary reactive amyloidosis, which was associated with rheumatoid arthritis (RA)...
  73. ncbi High-dose melphalan versus melphalan plus dexamethasone for AL amyloidosis
    Arnaud Jaccard
    Department of Hematology, Centre Hospitalier Universitaire, Université et Centre National de la Recherche Scientifique, UMR 6101, Limoges, France
    N Engl J Med 357:1083-93. 2007
    ..has been reported to provide higher response rates and better overall survival than standard chemotherapy in immunoglobulin-light-chain (AL) amyloidosis, but these two strategies have not been compared in a randomized study.
  74. ncbi Bone marrow angiogenesis in 400 patients with monoclonal gammopathy of undetermined significance, multiple myeloma, and primary amyloidosis
    S Vincent Rajkumar
    Division of Hematology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA
    Clin Cancer Res 8:2210-6. 2002
    ..To determine whether bone marrow (BM) angiogenesis progressively increases along the spectrum of plasma cell disorders ranging from monoclonal gammopathy of undetermined significance (MGUS) to advanced myeloma...
  75. pmc In vivo fragmentation of heparan sulfate by heparanase overexpression renders mice resistant to amyloid protein A amyloidosis
    Jin ping Li
    Department of Medical Biochemistry and Microbiology, Biomedical Center, Uppsala University, Box 582, SE 751 23 Uppsala, Sweden
    Proc Natl Acad Sci U S A 102:6473-7. 2005
    Amyloid diseases encompass >20 medical disorders that include amyloid protein A (AA) amyloidosis, Alzheimer's disease, and type 2 diabetes...
  76. ncbi Association of melphalan and high-dose dexamethasone is effective and well tolerated in patients with AL (primary) amyloidosis who are ineligible for stem cell transplantation
    Giovanni Palladini
    Amyloid Center Biotechnology Research Laboratories, Department of Biochemistry, University Hospital, Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy
    Blood 103:2936-8. 2004
    The most efficient therapeutic approach for immunoglobulin light chain amyloidosis (AL) is autologous stem cell transplantation (ASCT); however, the toxicity of ASCT limits its feasibility to a minority of patients...
  77. ncbi Dysferlin-deficient muscular dystrophy features amyloidosis
    Simone Spuler
    Muscle Research Unit, Experimental and Clinical Research Center at the Charité and the Max Delbrück Center, Berlin, Germany
    Ann Neurol 63:323-8. 2008
    ..Dysferlin (DYSF) gene mutations cause limb girdle muscular dystrophy type 2B and Miyoshi's myopathy. The consequences of DYSF mutations on protein structure are poorly understood...
  78. ncbi Detection of age-dependent brain injury in a mouse model of brain amyloidosis associated with Alzheimer's disease using magnetic resonance diffusion tensor imaging
    Shu Wei Sun
    Department of Radiology, Washington University School of Medicine, St Louis, MO 63130, USA
    Exp Neurol 191:77-85. 2005
    ..This work demonstrates the potential for DTI as a noninvasive modality to detect evolving pathology associated with changes in tissue water diffusion properties in brain tissues...
  79. ncbi Inhibition of Alzheimer's amyloidosis by peptides that prevent beta-sheet conformation
    C Soto
    Department of Neurology, New York University Medical Center, New York 10016, USA
    Biochem Biophys Res Commun 226:672-80. 1996
    ..The latter are more resistant to proteolytic degradation and may serve as a starting point to design more efficient peptides derivatives to inhibit amyloidogenesis in vivo...
  80. ncbi Transthyretin amyloidosis: a tale of weak interactions
    M J Saraiva
    Amyloid Unit, Instituto Biologia Molecular e Celular, and Instituto de Ciências Biomédicas, Universidade do Porto, R Campo Alegre, 823, 4150, Porto, Portugal
    FEBS Lett 498:201-3. 2001
    ..Avoiding tetramer dissociation and disrupting amyloid fibrils are possible avenues of therapeutic intervention based on current molecular knowledge of TTR amyloidogenesis and fibril structure...
  81. pmc The tumor-necrosis-factor receptor-associated periodic syndrome: new mutations in TNFRSF1A, ancestral origins, genotype-phenotype studies, and evidence for further genetic heterogeneity of periodic fevers
    I Aksentijevich
    Section of Genetics, Arthritis and Rheumatism Branch, National Institutes of Health, Bethesda, MD 20892, USA
    Am J Hum Genet 69:301-14. 2001
    ..sterile peritonitis, arthralgia, myalgia, skin rash, and/or conjunctivitis; some patients also develop systemic amyloidosis. Elsewhere we have described six disease-associated TNFRSF1A mutations, five of which disrupt extracellular ..
  82. ncbi Long-term in vivo imaging of β-amyloid plaque appearance and growth in a mouse model of cerebral β-amyloidosis
    Jasmin K Hefendehl
    Department of Cellular Neurology, Hertie Institute for Clinical Brain Research, University of Tubingen, Tubingen, Germany
    J Neurosci 31:624-9. 2011
    ..At later time points (i.e., in the presence of increasing cerebral β-amyloidosis), the number of newly formed plaques decreased...
  83. pmc Beta2-microglobulin isoforms display an heterogeneous affinity for type I collagen
    Sofia Giorgetti
    Dipartimento di Biochimica, Universita degli Studi di Pavia, via Taramelli 3 b 27100 Pavia, Italy
    Protein Sci 14:696-702. 2005
    ....
  84. ncbi The spectrum of localized amyloidosis: a case series of 20 patients and review of the literature
    Michelle L Biewend
    Mayo Clinic, Jacksonville, FL, USA
    Amyloid 13:135-42. 2006
    ..We identified 20 cases of localized amyloidosis at our institution between 1993 and 2003. There were 11 males and nine females in the group...
  85. ncbi High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study
    Martha Skinner
    Boston University School of Medicine, Boston, Massachusetts 02118, USA
    Ann Intern Med 140:85-93. 2004
    AL amyloidosis is a fatal disease resulting from tissue deposition of amyloid fibrils derived from monoclonal immunoglobulin light chains. Treatment with oral chemotherapy is minimally effective.
  86. ncbi Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosis
    Helen J Lachmann
    National Amyloidosis Centre, Department of Medicine, Royal Free and University College Medical School, Royal Free Campus, London, United Kingdom
    N Engl J Med 346:1786-91. 2002
    Hereditary, autosomal dominant amyloidosis, caused by mutations in the genes encoding transthyretin, fibrinogen A alpha-chain, lysozyme, or apolipoprotein A-I, is thought to be extremely rare and is not routinely included in the ..
  87. pmc Association of aortic atherosclerosis with cerebral beta-amyloidosis and learning deficits in a mouse model of Alzheimer's disease
    Ling Li
    Departments of Medicine, Pharmacology, and Genetics, University of Alabama at Birmingham, Birmingham, Alabama 35294, USA
    Am J Pathol 163:2155-64. 2003
    High fat/high cholesterol diets exacerbate beta-amyloidosis in mouse models of Alzheimer's disease (AD)...
  88. pmc SCFFbx2-E3-ligase-mediated degradation of BACE1 attenuates Alzheimer's disease amyloidosis and improves synaptic function
    Bing Gong
    Department of Neurology, Mount Sinai School of Medicine, New York, NY 10029, USA
    Aging Cell 9:1018-31. 2010
    ..Our study is the first to suggest that promoting Fbx2 in the brain may represent a novel strategy for the treatment of AD...
  89. pmc Pancreatic islet amyloidosis, beta-cell apoptosis, and alpha-cell proliferation are determinants of islet remodeling in type-2 diabetic baboons
    Rodolfo Guardado-Mendoza
    Department of Medicine Diabetes Division, University of Texas Health Science Center, San Antonio, TX 78229, USA
    Proc Natl Acad Sci U S A 106:13992-7. 2009
    ..is an important factor in the development of hyperglycemia of type-2 diabetes mellitus, and pancreatic islet amyloidosis (IA) has been postulated to be one of the main contributors to impaired insulin secretion...
  90. ncbi Familial Mediterranean fever: clinical, molecular and management advancements
    M Lidar
    Heller Institute of Medical Research, Sheba Medical Centre, Tel Hashomer, affiliated with the Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
    Neth J Med 65:318-24. 2007
    ..frequency, duration and intensity in the majority of patients, and also prevents the development of secondary amyloidosis, the most dreaded complication of the disease...
  91. ncbi Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens
    Julie A Vrana
    Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA
    Blood 114:4957-9. 2009
    The clinical management of amyloidosis is based on the treatment of the underlying etiology, and accurate identification of the protein causing the amyloidosis is of paramount importance...
  92. ncbi Targeted pharmacological depletion of serum amyloid P component for treatment of human amyloidosis
    M B Pepys
    Centre for Amyloidosis and Acute Phase Proteins, Department of Medicine, Royal Free and University College Medical School, London NW3 2PF, UK
    Nature 417:254-9. 2002
    ..P component (SAP) binds to fibrils in all types of amyloid deposits, and contributes to the pathogenesis of amyloidosis. In order to intervene in this process we have developed a drug, R-1-[6-[R-2-carboxy-pyrrolidin-1-yl]-6-oxo-..
  93. ncbi Mapping the core of the beta(2)-microglobulin amyloid fibril by H/D exchange
    Masaru Hoshino
    Institute for Protein Research, Osaka University, Yamadaoka 3 2, Suita, Osaka 565 0871, Japan
    Nat Struct Biol 9:332-6. 2002
    ..The present method could be used to obtain residue-specific conformational information of various amyloid fibrils, even though it does not provide a high resolution three-dimensional structure...
  94. ncbi Repetitive mild brain trauma accelerates Abeta deposition, lipid peroxidation, and cognitive impairment in a transgenic mouse model of Alzheimer amyloidosis
    Kunihiro Uryu
    Department of Pathology and Laboratory Medicine, Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 4283, USA
    J Neurosci 22:446-54. 2002
    ..insights into the role of TBI in mechanisms of AD pathobiology could lead to strategies for reducing the risk of AD associated with previous episodes of brain trauma and for preventing progressive brain amyloidosis in AD patients.
  95. ncbi Clinical aspects of systemic amyloid diseases
    Laura Obici
    Amyloid Center, Biotechnology Research Laboratories, IRCCS Policlinico San Matteo, Pavia, Italy
    Biochim Biophys Acta 1753:11-22. 2005
    b>Amyloidosis is a protein misfolding disorder in which soluble proteins aggregate as insoluble amyloid fibrils. Protein aggregates and amyloid fibrils cause functional and structural organ damage respectively...
  96. pmc Role of endocytic inhibitory drugs on internalization of amyloidogenic light chains by cardiac fibroblasts
    Grace Fortes Monis
    Department of Pathology, Gerry Amyloid Research Laboratory, Boston University School of Medicine, 80 E Concord St L904, Boston, MA 02118, USA
    Am J Pathol 169:1939-52. 2006
    b>Amyloidosis is a disease of protein misfolding that ultimately impairs organ function...
  97. ncbi Folding proteins in fatal ways
    Dennis J Selkoe
    Center for Neurologic Diseases, Harvard Medical School, Brigham and Women s Hospital, Boston, Massachusetts 02115, USA
    Nature 426:900-4. 2003
    ..Understanding some of the principles of protein folding has helped to explain how such diseases arise, with attendant therapeutic insights...
  98. ncbi Serum N-terminal pro-brain natriuretic peptide is a sensitive marker of myocardial dysfunction in AL amyloidosis
    Giovanni Palladini
    Department of Internal Medicine, University Hospital IRCCS Policlinico San Matteo University of Pavia, Pavia, Italy
    Circulation 107:2440-5. 2003
    ..involvement is the most important prognostic factor and causes death in almost all patients with light-chain amyloidosis (AL). We investigated the prognostic value of NT-proBNP and its utility in monitoring amyloid heart dysfunction.
  99. ncbi Human amyloidogenic light chains directly impair cardiomyocyte function through an increase in cellular oxidant stress
    Daniel A Brenner
    Whitaker Cardiovascular Institute and Amyloid Treatment and Research Program, Evans Department of Medicine, Boston University School of Medicine, Boston, Mass 02118, USA
    Circ Res 94:1008-10. 2004
    Primary amyloidosis is a systemic disorder characterized by the clonal production and tissue deposition of immunoglobulin light chain (LC) proteins...
  100. ncbi Ibuprofen suppresses interleukin-1beta induction of pro-amyloidogenic alpha1-antichymotrypsin to ameliorate beta-amyloid (Abeta) pathology in Alzheimer's models
    Takashi Morihara
    Greater Los Angeles VA Healthcare System, GRECC, Sepulveda, CA, USA
    Neuropsychopharmacology 30:1111-20. 2005
    ..We present evidence supporting the hypothesis that ibuprofen-dependent amyloid reduction is mediated by inhibition of an alternate pathway (IL-1beta and its downstream target ACT)...
  101. ncbi Collagen plays an active role in the aggregation of beta2-microglobulin under physiopathological conditions of dialysis-related amyloidosis
    Annalisa Relini
    Department of Physics, University of Genoa, Via Dodecaneso 33, I 16146 Genoa, Italy
    J Biol Chem 281:16521-9. 2006
    Dialysis-related amyloidosis is characterized by the deposition of insoluble fibrils of beta(2)-microglobulin (beta(2)-m) in the musculoskeletal system...

Research Grants74

  1. Reactive (AA)Amyloidosis
    Merrill D Benson; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): AA amyloidosis (reactive, secondary) is caused by the extracellular deposition of protein fibrils which disrupts normal organ function and usually leads to death from renal, hepatic or cardiac ..
  2. Vascular dysfunction and oxidative stress in primary amyloidosis
    RAYMOND QUEZON MIGRINO; Fiscal Year: 2010
    DESCRIPTION (provided by applicant): Primary systemic amyloidosis is a plasma cell dyscrasia that is often fatal especially if there is cardiac involvement...
  3. Systemic AA Amyloidosis Inhibitors
    ALAN D SNOW; Fiscal Year: 2010
    DESCRIPTION (provided by applicant): Systemic AA Amyloidosis is characterized by the accumulation of insoluble fibril deposits containing the AA amyloid protein in different organs throughout the body including heart, kidney, liver, ..
  4. Beta Cell Adaptation to Stress in Baboon Pancreas After Partial Pancreatectomy
    FRANCO BATTISTA FOLLI; Fiscal Year: 2010
    ..role of amyloidogenic peptides in [unreadable]-cell decompensation, because NHPs spontaneously develop islet amyloidosis and T2DM very similarly to humans...
  5. Improving CBF through direct control of CSF pulsations
    MARK GREGORY LUCIANO; Fiscal Year: 2010
    ..with diminished CBF including Alzheimer's and vascular type dementia, vascular diseases such as vasospasm, amyloidosis, atherosclerosis, venous hypertension, and CSF hydrodynamic disorders such as hydrocephalus and pseurdotumor ..
  6. Regulation of HMGB1 Release in Endotoxemia
    Haichao Wang; Fiscal Year: 2013
    ..accumulation, leukocyte infiltration and bacterial clearance, or hepatic injury from excessive autophagy or amyloidosis. The completion of these exciting studies will further improve our understanding of the mechanisms underlying ..
  7. Nanoliposome-based Treatment of Amyloid Protein (AL) Toxicity
    RAYMOND QUEZON MIGRINO; Fiscal Year: 2013
    ..provided by applicant): Amyloid protein-misfolding diseases such as Alzheimer's (AD) and light chain amyloidosis (AL) remain intractable diseases with high morbidity and mortality...
  8. Molecular Mechanisms and Treatment of Primary Amyloid AL Cardiomyopathy
    Ronglih Liao; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Amyloidosis represents a group of human diseases in which protein precursors exhibit altered secondary structure and form insoluble protein aggregates in tissues...
  9. The Abeta protein of Alzheimer's Disease is an antimicrobial peptide
    Robert D Moir; Fiscal Year: 2013
    ..If validated the identification of A[unreadable] as an antimicrobial agent raises the possibility of preventing amyloidosis from starting by preemptive targeting of pathogens/insults that stimulate the brain's innate immune system...
  10. MEMBRANE FUSION IN RETINAL ROD OUTER SEGMENTS
    KATHLEEN BOESZEBATTAGLIA; Fiscal Year: 2012
    ..number of IDD containing proteins are associated with human diseases, including cancer, cardiovascular disease, amyloidosis, neurodegenerative diseases and diabetes...
  11. Mechanism of CD40 signaling to APP metabolism
    Michael J Mullan; Fiscal Year: 2013
    ..inhibit CD40 downstream signaling molecules validated in vitro and in CD40 deficient models of cerebral amyloidosis and tau pathology characteristic of AD...
  12. Matrix and trophic coupling in the neurovascular unit
    Eng H Lo; Fiscal Year: 2011
    ..in vivo mouse models: focal cerebral ischemia, aging, and the APPswe/PS1dE9 transgenic mouse model of cerebral amyloidosis. We will use a combination of in vivo 2-photon imaging, laser capture microdissection, and standard ..
  13. Neuroprotective immunity and HIV dementia
    Howard E Gendelman; Fiscal Year: 2013
    ..associated, in measure, with atrophy of the nigrostriatum and frontostriatal circuits and early signs of beta-amyloidosis, microtubule-associated tau aggregation, and alpha synuclein inclusions...
  14. A Mouse Model for the Rare Plasma Cell Disease AL Amyloidosis
    David C Seldin; Fiscal Year: 2010
    ..In this application, we propose to develop a "second generation" transgenic mouse model of the rare disease AL amyloidosis. AL is a disease with features of a plasma cell dyscrasia, and is caused by a low grade clonal expansion of ..
  15. RAGE Mediated Mechanisms of Islet Amyloid Polypeptide Cytotoxicity in Type 2 Diab
    Andisheh Abedini; Fiscal Year: 2011
    ..for the treatment and prevention of T2D, but may also be translated to better controlling pathological amyloidosis associated with other diseases. PHS 398/2590 (Rev...
  16. Epigenomic impact of diet and toxicant exposure in Alzheimers disease etiology
    Iliya Lefterov; Fiscal Year: 2013
    ..The detrimental effects of high fat diet (HFD) on cognitive performance and exacerbation of cerebral amyloidosis and amyloid angiopathy has been recently demonstrated in an animal model of AD...
  17. Role of Presenilin in Idiopathic Dilated Cardiomyopathy
    Federica Del Monte; Fiscal Year: 2013
    ..misfolding of proteins is a common cause of many apparently unrelated chronic diseases such as primary systemic amyloidosis, diabetes, cystic fibrosis, neurodegenerative diseases and inclusion body myositis...
  18. A Proof of Concept Study Examining The Safety and Efficacy of Innate Immune Syste
    Ted T Ashburn; Fiscal Year: 2013
    ..of sargramostim should allow for therapeutic activity in the CNS, (6) murine GM-CSF (mGM-CSF) reverses both amyloidosis and cognitive impairment in AD mice, (7) the safety profile of sargramostim allows for its long-term use in non-..
  19. Aging and brain 5-lipoxygenase
    Hari Manev; Fiscal Year: 2010
    ..have shown that 5-LOX knockout in a mouse model of Alzheimer's disease, the Tg2576 mouse, and decreases amyloidosis. Recent preliminary data indicate that neuronal 5-LOX expression increases following changes in the methylation ..
  20. Genetic analysis and regulation of amyloids flux in the Drosophila heart
    GIRISH CHANDRA MELKANI; Fiscal Year: 2011
    ..Despite several proteins being shown to be associated with cardiac amyloidosis, the precise mechanism that leads to the disease is poorly known...
  21. Investigation of the Establishment of the Prion State
    Irina L Derkatch; Fiscal Year: 2010
    ..Comparative analysis of prions and other proteins linked to human amyloidosis, and the identification of prions among benign epigenetic modifiers of cellular functions in fungi suggest the ..
  22. Inhibitors of IgH Gene Enhancers for the Treatment of Multiple Myeloma
    FRANK W MARCOUX; Fiscal Year: 2013
    ..just MM but nearly all B and plasma cell malignancies, including non-Hodgkin lymphoma, plasma cell leukemia, amyloidosis, and others...
  23. In Vivo Reconstitution Models for NeuroAIDS and Beta-Amyloidosis
    Tsuneya Ikezu; Fiscal Year: 2010
    ..We propose developing animal models of beta-amyloidosis in HIV infected brains...
  24. PROTEINS IN MULTIPLE MYELOMA AND RELATED BLOOD DISORDERS
    Alan Solomon; Fiscal Year: 2011
    DESCRIPTION (provided by applicant): Primary systemic amyloidosis, i.e...
  25. Role of the MyD88-independent pathway in Alzheimers disease
    Ken Ichiro Fukuchi; Fiscal Year: 2013
    ..Therefore, we hypothesize that the TLR4-mediated MyD88-dependent pathway exacerbates cerebral [unreadable]-amyloidosis in vivo and that TLR4-mediated MyD88-independent (TRIF-dependent) signaling pathways play a significant role in ..
  26. The Role of Oxidative Stress in Systemic Amyloidosis
    Steven R Zeldenrust; Fiscal Year: 2010
    ..seeks to bring his skills and expertise to bear on the role of oxidative stress in the pathogenesis of systemic amyloidosis. This project builds on the candidate's prior experience in basic research in hereditary amyloidosis, ..
  27. Identification of Novel Small Molecules as Tau Protein Aggregation Inhibitors for
    ALAN D SNOW; Fiscal Year: 2011
    ..of different in vitro screening technologies and cellular models that identified new and potent inhibitors of amyloidosis. The techniques led us to design and synthesize a class of [new chemical entities consisting of small molecules ..
  28. Implication of HUCBC: a novel therapeutic strategy for Alzheimer's disease
    Jun Tan; Fiscal Year: 2013
    ..Here we propose to test the hypothesis that HUCBC mediated dampening of the CD40-CD40L interaction reduces amyloidosis by investigating CD40 signaling in HUCBC infused PSAPP mice...
  29. Genetic analysis and regulation of amyloids flux in the Drosophila heart
    GIRISH CHANDRA MELKANI; Fiscal Year: 2012
    ..Despite several proteins being shown to be associated with cardiac amyloidosis, the precise mechanism that leads to the disease is poorly known...
  30. A role for IL-1 in SJIA monocyte phenotypes: A RAPPORT ancillary study
    Elizabeth D Mellins; Fiscal Year: 2013
    ..Although the etiology is unknown, SJIA complications such as macrophage activation syndrome and amyloidosis support involvement of the innate immune system, as do the paucity of autoantibodies or MHC alleles that ..
  31. Novel Insights into the Pathogenesis of Light Chain Cardiac Amyloidosis
    Flora Sam; Fiscal Year: 2011
    DESCRIPTION (provided by applicant): Systemic amyloidosis is a rare disease that affects many organ systems, the most devastating and lethal of which is cardiac involvement. In response to statements from the U.S...
  32. Molecular mechanism of senile cardiac amyloidosis
    Lawreen H Connors; Fiscal Year: 2013
    ..result of extracellular deposits of amyloid in the heart, pathology referred to as Senile Systemic (or Cardiac) Amyloidosis (SSA)...
  33. Peripheral TGF-beta Pathway Inhibitor Therapy in Alzheimer's Rats
    Terrence Town; Fiscal Year: 2013
    ..elderly controls, potentially biasing toward a suppressive milieu that is ineffective at restricting cerebral amyloidosis. Our published and preliminary data using genetic and pharmacologic approaches in mouse models of cerebral ..
  34. Caloric restriction and Alzheimers ABeta clearance pathway
    Berislav V Zlokovic; Fiscal Year: 2013
    ..A[unreadable]) and on cognitive functions using a transgenic model of AD-like cerebral [unreadable]-amyloidosis. The proposed research will significantly enhance the neuroscience research capacity at the LMIC site which is ..
  35. The Neurobiology of 5-Lipoxygenase
    Domenico Pratico; Fiscal Year: 2013
    ..in wild type mice, and significantly less A[unreadable] deposits in the Tg2576 mice, a mouse model of AD-like amyloidosis. Further, in cell culture systems 5LO activation and pharmacologic inhibition results in increased and reduced ..
  36. Aging and the Tissue Response to Misfolded Proteins
    Joel N Buxbaum; Fiscal Year: 2012
    ..Alzheimers Disease, type II diabetes and senile systemic amyloidosis are the result of tissue deposition of the wild type protein precursors A2, islet amyloid polypeptide and ..
  37. Console for an 800/89 DNP Spectrometer
    ROBERT GUY GRIFFIN; Fiscal Year: 2013
    ..phosphatidyl-inositol-3-kinase SH3 domain (PI3- SH3), (7) beta-2-microglobulin associated with dialysis related amyloidosis, and (8) the CsgA protein, from Curli...
  38. Metabolite Initiated Protein Misfolding and Amyloidosis
    Jeffery W Kelly; Fiscal Year: 2013
    ..abstract_text> ..
  39. Preamyloid Oligomers and Susceptibility to Atrial Fibrillation
    Katherine T Murray; Fiscal Year: 2013
    ..Unexpectedly, we observed conserved transcriptional upregulation in proteins involved in amyloidosis, a process associated with protein misfolding and deposition in multiple neurodegenerative diseases, notably ..
  40. Targeting Abeta phagocytosis by blocking IRAK-M innate immunity in Alzheimer mice
    David Gate; Fiscal Year: 2013
    ..with mice over expressing mutant human amyloid precursor protein, the APPPS1 mouse model of cerebral amyloidosis. We then evaluated the state of immune cell activation and cerebral A[unreadable] burden in age and sex- ..
  41. Beta-amyloid Modulation: Role of BACE1/BACE2
    Philip C Wong; Fiscal Year: 2010
    ..secretase, these two enzymes represent high priority targets for drug discoveries in efforts to attenuate A amyloidosis in AD...
  42. Mechanisms of fibril formation in light chain amyloidosis
    DOUGLAS J MARTIN; Fiscal Year: 2011
    Light chain amyloidosis (AL) is a hematological disorder that occurs when a clonal population of B cells overproduces immunoglobulin light chains...
  43. Cerebrovascular beta-Amyloidosis: A-beta CNS Transport Pathways
    Berislav V Zlokovic; Fiscal Year: 2013
    ..RAGE mediates AI3 suppression of cerebral blood flow, neurovascular stress and development of cerebral 13-amyloidosis. Treatment of Tg PD hAPP mice with soluble RAGE (sRAGE) decreases the CNS amyloid load and AI3 levels, and ..
  44. Engineering chaperones for extracellular amyloids
    Diego E Rincon-Limas; Fiscal Year: 2013
    ..are expected to have a positive impact because they will lead to the application of secHsp70 in other cerebral amyloidosis, such as prion diseases, as well as in disorders with systemic distribution of amyloids, such as Type 2 ..
  45. Intraneuronal Abeta accumulation: mechanism of pathogenesis
    Gunnar K Gouras; Fiscal Year: 2010
    ..immuno-gold electron microscopy, we reported in transgenic mutant APR mice that develop age-related beta-amyloidosis the accumulation of beta-amyloid especially in late endosomal vesicles of distal processes and synaptic ..
  46. Amyloid ion channels to design therapeutics for neurodegenerative diseases
    Ratneshwar Lal; Fiscal Year: 2013
    ..Parkinson's, familial dementia, prion encephalopathies), systemic (type II diabetes, light chain amyloidosis related cancer) and other (cystic fibrosis) diseases...
  47. BIOLOGY OF ALZHEIMER AMYLOID IN NEURONS
    Gunnar K Gouras; Fiscal Year: 2010
    ..in the brain and to determine how plaques form in a well-established transgenic mouse model of beta-amyloidosis. We found that beta-amyloid localized especially to small intracellular organelles, called multivesicular ..
  48. Preclinical Diagnostic Imaging of Amyloid
    Jonathan S Wall; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Amyloidosis is a devastating pathology associated with a growing number of diseases, including two of the most socio-economically impacting conditions of our time, Alzheimer's disease (Abeta ..
  49. Insulin resistance, amyloid, and memory in normal aging and preclinical AD
    JILL KATHLEEN MORRIS; Fiscal Year: 2013
    ..By investigating insulin sensitivity in subjects with or without cerebral amyloidosis, we will determine whether these factors influence insulin- mediated cognitive improvement in the very earliest ..
  50. 800 MHz Low Temperature Magic Angle Spinning NMR Probe.
    Robert Griffin; Fiscal Year: 2009
    ..phosphatidyl inositol-3-kinase SH3 domain (PI3-SH3), (6) beta-2-microglobulin associated with dialysis related amyloidosis, and (7) the Sup35p protein, a yeast prion system...
  51. I2PP2A: A Therapeutic Target
    Khalid Iqbal; Fiscal Year: 2013
    ..whether knockdown of I2PP2A can inhibit abnormal hyperphosphorylation of tau/neurofibrillary degeneration and b-amyloidosis in 3xTgAD mice;and (2) To study whether knockdown of I2PP2A can inhibit neurodegeneration and associated ..
  52. Lipid acceleration of transthyretin amyloidosis
    Irit Rappley; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): The transthyretin (TTR) amyloidoses, including senile systemic amyloidosis, familial amyloid cariomyopathy, and familial amyloid polyneuropathy, are characterized by extracellular amyloid deposits ..
  53. The functional role of FLAP in Alzheimer's Disease
    Domenico Pratico; Fiscal Year: 2012
    ..If we demonstrate that MK-591 administration results in a modulation (decrease) of brain amyloidosis, and improvement of behavioral impairments in this AD model, our findings will represent the biologic basis for ..
  54. Cerebral Amyloidosis and Dementia
    JORGE A GHISO; Fiscal Year: 2013
    ..abstract_text> ..
  55. Oral administration of TeaMemTM inhibits Alzheimer's disease pathogenesis
    Jun Tan; Fiscal Year: 2009
    ..In this proposal, we will evaluate EGCG's therapeutic effect on reducing cerebral amyloidosis and improving cognitive impairment in a transgenic mouse model of AD via an oral administration...
  56. Radioimmunoimaging of AL Amyloidosis using 124I-mAb 11-1F4 (Phase I/IND 100472/1-
    Alan Solomon; Fiscal Year: 2010
    ..antibody Mu 11-1F4 labeled with I-124 to target amyloid deposits in organs or tissues of patients with AL amyloidosis. AL amyloidosis, a monoclonal plasma cell dyscrasia, is an illness associated with considerable morbidity that ..
  57. Imaging AD Amyloidosis Pathology by Novel Multi-modal NIR/PET/19F MRI Probes
    Chongzhao Ran; Fiscal Year: 2013
    ..If successful, with an arsenal of probes for detection of both insoluble plaques and soluble A2 species, we believe that eventually we will be able to monitor the full course of amyloidosis pathology of Alzheimer's disease.
  58. Mechanisms of Aggregation in Light Chain Amyloidosis
    Marina Ramirez-Alvarado; Fiscal Year: 2013
    ..The overall goal of this proposal is to identify the misfolded cytotoxic species populated by light chain amyloidosis (AL) proteins...
  59. Cardiac Amyloidosis in Aging African American
    Joel N Buxbaum; Fiscal Year: 2012
    ..During the last four years we have shown that a genetically determined form of late-onset amyloidosis due to a substitution of ILE for VAL at position 122 in the serum protein transthyretin (TTR) contributes to ..
  60. Late Gadolinium Enhancement MRI for Patients with Implantable Cardiac Devices
    Peng Hu; Fiscal Year: 2013
    ..forms of non-ischemic cardiomyopathy, including myocarditis, arrhythmogenic right ventricular dysplasia, amyloidosis, and sarcoidosis...
  61. Probing the biochemical mechanism of amyloid disease
    Jeffery W Kelly; Fiscal Year: 2013
    ..the liver, and since liver transplantation from an amyloid patient into a liver cancer patient initiates rapid amyloidosis in the latter, we are confident that studying liver physiology in amyloidosis, and normal young and old donors ..
  62. Octyl gallate (OG) and Promotion of non-amyloidogenic processing of APP
    Jun Tan; Fiscal Year: 2013
    ..opposing cleavage of amyloid precursor protein (APP) into A[unreadable] peptides and resultant cerebral amyloidosis, a key pathological feature of AD, have become a primary focus in recent years...
  63. Phase II, IL-1 Trap for Treatment of Familial Mediterranean Fever
    Steven J Spalding; Fiscal Year: 2010
    ..Late complications of untreated FMF include the development of renal amyloidosis. FMF is a rare orphan disease in the United States...
  64. Systemic AA Amyloidosis Inhibitors
    ALAN SNOW; Fiscal Year: 2004
    Systemic AA amyloidosis is characterized by the deposition and accumulation of insoluble fibrillar deposits containing the AA amyloid protein in a number of different organs (including heart, kidney, spleen, lungs and/or ..
  65. Biogenesis and Function of Bacterial Amyloid fibers
    Matthew Chapman; Fiscal Year: 2005
    ..amyloid fibers that are involved in several mammalian ailments including Alzheimer's disease, systemic amyloidosis, and spongiform encephalopathies such as mad cow disease and Creutzfeldt-Jacob disease...
  66. IMMUNOGLOBULINS IN MULTIPLE MYELOMA & AMYLOIDOSIS
    Allen Edmundson; Fiscal Year: 1993
    ..from the sera (IgG and IgM immunogobulins) and urines (Bence-Jones proteins) of patients with multiple myeloma, amyloidosis and Waldenstrom's macroglobulinemia...
  67. CEREBROVASCULAR AMYLOIDOSIS
    Peter Gorevic; Fiscal Year: 1999
    ..Forms with a predilection for the central nervous system that may manifest prominent cerebrovascular amyloidosis include the prion diseases, certain hereditary stroke-like syndromes due to cystatin C or Alzheimer's disease (..
  68. METABOLISM OF SERUM AMYLOID A (SAA:HDL)
    Barbara Kluve Beckerman; Fiscal Year: 2007
    The overall goal of this project is to elucidate pathogenic mechanisms of reactive (AA) amyloidosis. This type of amyloidosis develops as a complication of chronic and recurrent inflammatory conditions such as rheumatoid arthritis and ..
  69. Alzheimers Disease Mechanism & Experimental Therapeutic
    Philip Wong; Fiscal Year: 2009
    ..but also allow a critical evaluation of these proteins as therapeutic targets in efforts to ameliorate Abeta amyloidosis in individuals with AD. PROJECT 1 P.I.: Donald L. Price, M.D...
  70. Anti-inflammatory regulation of beta-amyloidosis
    Tsuneya Ikezu; Fiscal Year: 2010
    ..is to determine the regulatory role of anti-inflammatory molecules on neuroinflammatory activities and beta-amyloidosis (A[unreadable] aggregation and deposition in brain) in rodent models of Alzheimer's disease (AD)...
  71. MOLECULAR ASPECTS OF AMYLOIDOSIS
    WARREN LIAO; Fiscal Year: 1999
    ..short-term survival function, prolonged elevation of plasma SAA protein, however, will lead to secondary amyloidosis, an incurable disease...
  72. BIOMETALS, OXIDATIVE STRESS, AND AB AMYLOIDOSIS
    Xudong Huang; Fiscal Year: 2005
    ..enhances cellular APP expression, and heightens Abeta production, implicating a vicious cycle between Abeta amyloidosis and oxidative stress...
  73. Design & Synthesis of Novel Agents to Treat Amyloidosis
    Michael Flavin; Fiscal Year: 2003
    The major goal of our proposed project is to advance the development of novel compounds for the treatment of amyloidosis and other protein aggregation diseases...
  74. Transgenic Model for Cerebral Amyloid Angiopathy
    William Van Nostrand; Fiscal Year: 2006
    ..feature of patients with Alzheimer's disease (AD) and several related hereditary cerebral hemorrhage with amyloidosis (HCHWA) disorders...