inclusion body myositis

Summary

Summary: Progressive myopathies characterized by the presence of inclusion bodies on muscle biopsy. Sporadic and hereditary forms have been described. The sporadic form is an acquired, adult-onset inflammatory vacuolar myopathy affecting proximal and distal muscles. Familial forms usually begin in childhood and lack inflammatory changes. Both forms feature intracytoplasmic and intranuclear inclusions in muscle tissue. (Adams et al., Principles of Neurology, 6th ed, pp1409-10)

Top Publications

  1. ncbi Proteasomal expression, induction of immunoproteasome subunits, and local MHC class I presentation in myofibrillar myopathy and inclusion body myositis
    Isidro Ferrer
    Instituto de Neuropatologia, Servicio de Anatomia Patologica, Hospital Universitario de Bellvitge, Hospitalet de Llobregat, Spain
    J Neuropathol Exp Neurol 63:484-98. 2004
  2. ncbi Inclusion body myositis: current pathogenetic concepts and diagnostic and therapeutic approaches
    Merrilee Needham
    Centre for Neuromuscular and Neurological Disorders, University of Western Australia, Queen Elizabeth II Medical Centre, Perth, Australia
    Lancet Neurol 6:620-31. 2007
  3. pmc Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis
    Mohammad Salajegheh
    Department of Neurology, Division of Neuromuscular Disease, Brigham and Women s Hospital, and Harvard Medical School, 75 Francis Street, Boston, Massachusetts 02115, USA
    Muscle Nerve 40:19-31. 2009
  4. pmc Sporadic inclusion body myositis: possible pathogenesis inferred from biomarkers
    Conrad C Weihl
    Department of Neurology and Hope Center for Neurological Disorders, Washington University School of Medicine, 660 S Euclid Avenue, Saint Louis, Missouri 63110, USA
    Curr Opin Neurol 23:482-8. 2010
  5. ncbi Inclusion body myositis: a degenerative muscle disease associated with intra-muscle fiber multi-protein aggregates, proteasome inhibition, endoplasmic reticulum stress and decreased lysosomal degradation
    Valerie Askanas
    USC Neuromuscular Center, Department of Neurology, University of Southern California Keck School of Medicine, Good Samaritan Hospital, Los Angeles, CA 90017 1912, USA
    Brain Pathol 19:493-506. 2009
  6. ncbi Prevalence of sporadic inclusion body myositis and factors contributing to delayed diagnosis
    Merrilee Needham
    Centre for Neuromuscular and Neurological Disorders, Australian Neuromuscular Research Institute ANRI, University of Western Australia, Queen Elizabeth II Medical Centre, Nedlands, Perth 6009, Australia
    J Clin Neurosci 15:1350-3. 2008
  7. ncbi Epidemiology of sporadic inclusion body myositis and polymyositis in Olmsted County, Minnesota
    Floranne C Wilson
    Division of Rheumatology, Mayo Clinic, College of Medicine, Rochester, Minnesota 55905, USA
    J Rheumatol 35:445-7. 2008
  8. ncbi Expanded T cell receptor Vβ-restricted T cells from patients with sporadic inclusion body myositis are proinflammatory and cytotoxic CD28null T cells
    Jayesh M Pandya
    Rheumatology Unit, Karolinska University Hospital, Stockholm, Sweden
    Arthritis Rheum 62:3457-66. 2010
  9. pmc Inclusion-body myositis: muscle-fiber molecular pathology and possible pathogenic significance of its similarity to Alzheimer's and Parkinson's disease brains
    Valerie Askanas
    Department of Neurology, USC Neuromuscular Center, Good Samaritan Hospital, University of Southern California Keck School of Medicine, 637 South Lucas Avenue, Los Angeles, CA 90017 1912, USA
    Acta Neuropathol 116:583-95. 2008
  10. ncbi Immunolocalization of tumor necrosis factor-alpha and its receptors in inflammatory myopathies
    J L De Bleecker
    Neurology Department, University Hospital, Gent, Belgium
    Neuromuscul Disord 9:239-46. 1999

Detail Information

Publications245 found, 100 shown here

  1. ncbi Proteasomal expression, induction of immunoproteasome subunits, and local MHC class I presentation in myofibrillar myopathy and inclusion body myositis
    Isidro Ferrer
    Instituto de Neuropatologia, Servicio de Anatomia Patologica, Hospital Universitario de Bellvitge, Hospitalet de Llobregat, Spain
    J Neuropathol Exp Neurol 63:484-98. 2004
    b>Inclusion body myositis (IBM) and myofibrillar myopathy (MM) are diseases characterized by the abnormal accumulation of proteins in muscle fibers, including desmin, alphaB-crystallin, gelsolin, actin, kinases, and phospho-tau, along with ..
  2. ncbi Inclusion body myositis: current pathogenetic concepts and diagnostic and therapeutic approaches
    Merrilee Needham
    Centre for Neuromuscular and Neurological Disorders, University of Western Australia, Queen Elizabeth II Medical Centre, Perth, Australia
    Lancet Neurol 6:620-31. 2007
    b>Inclusion body myositis is the most common acquired muscle disease in older individuals, and its prevalence varies among countries and ethnic groups...
  3. pmc Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis
    Mohammad Salajegheh
    Department of Neurology, Division of Neuromuscular Disease, Brigham and Women s Hospital, and Harvard Medical School, 75 Francis Street, Boston, Massachusetts 02115, USA
    Muscle Nerve 40:19-31. 2009
    ..nucleic acid binding protein TDP-43 was recently identified in normal myonuclei and in the sarcoplasm of inclusion body myositis (IBM) muscle...
  4. pmc Sporadic inclusion body myositis: possible pathogenesis inferred from biomarkers
    Conrad C Weihl
    Department of Neurology and Hope Center for Neurological Disorders, Washington University School of Medicine, 660 S Euclid Avenue, Saint Louis, Missouri 63110, USA
    Curr Opin Neurol 23:482-8. 2010
    The relevance of proteins that accumulate and aggregate in the muscle fibers of patients with sporadic inclusion body myositis (sIBM) is unknown...
  5. ncbi Inclusion body myositis: a degenerative muscle disease associated with intra-muscle fiber multi-protein aggregates, proteasome inhibition, endoplasmic reticulum stress and decreased lysosomal degradation
    Valerie Askanas
    USC Neuromuscular Center, Department of Neurology, University of Southern California Keck School of Medicine, Good Samaritan Hospital, Los Angeles, CA 90017 1912, USA
    Brain Pathol 19:493-506. 2009
    Sporadic inclusion body myositis (s-IBM), the most common muscle disease of older persons, is of unknown cause, and there is no enduring treatment...
  6. ncbi Prevalence of sporadic inclusion body myositis and factors contributing to delayed diagnosis
    Merrilee Needham
    Centre for Neuromuscular and Neurological Disorders, Australian Neuromuscular Research Institute ANRI, University of Western Australia, Queen Elizabeth II Medical Centre, Nedlands, Perth 6009, Australia
    J Clin Neurosci 15:1350-3. 2008
    The prevalence of sporadic inclusion body myositis (sIBM) is variable in different populations and ethnic groups. A previous survey in Western Australia in 2000 found a prevalence of 9.3 per million population...
  7. ncbi Epidemiology of sporadic inclusion body myositis and polymyositis in Olmsted County, Minnesota
    Floranne C Wilson
    Division of Rheumatology, Mayo Clinic, College of Medicine, Rochester, Minnesota 55905, USA
    J Rheumatol 35:445-7. 2008
    To determine the incidence and prevalence of sporadic inclusion body myositis (sIBM) and polymyositis (PM) in a population-based study.
  8. ncbi Expanded T cell receptor Vβ-restricted T cells from patients with sporadic inclusion body myositis are proinflammatory and cytotoxic CD28null T cells
    Jayesh M Pandya
    Rheumatology Unit, Karolinska University Hospital, Stockholm, Sweden
    Arthritis Rheum 62:3457-66. 2010
    Sporadic inclusion body myositis (IBM) is characterized by T cell infiltrates in muscle tissue, but their functional role is unclear...
  9. pmc Inclusion-body myositis: muscle-fiber molecular pathology and possible pathogenic significance of its similarity to Alzheimer's and Parkinson's disease brains
    Valerie Askanas
    Department of Neurology, USC Neuromuscular Center, Good Samaritan Hospital, University of Southern California Keck School of Medicine, 637 South Lucas Avenue, Los Angeles, CA 90017 1912, USA
    Acta Neuropathol 116:583-95. 2008
    ..Similarities include, in the respective tissues, cellular aging, mitochondrial abnormalities, oxidative and endoplasmic-reticulum stresses, proteasome inhibition and multiprotein aggregates...
  10. ncbi Immunolocalization of tumor necrosis factor-alpha and its receptors in inflammatory myopathies
    J L De Bleecker
    Neurology Department, University Hospital, Gent, Belgium
    Neuromuscul Disord 9:239-46. 1999
    ..We immunolocalized TNF-alpha and its receptors in polymyositis, inclusion body myositis and dermatomyositis...
  11. pmc Endoplasmic reticulum stress and unfolded protein response in inclusion body myositis muscle
    Gaetano Vattemi
    Department of Neurology, University of Southern California Neuromuscular Center, University of Southern California Keck School of Medicine, Good Samaritan Hospital, Los Angeles, California 90017 1912, USA
    Am J Pathol 164:1-7. 2004
    ..containing either amyloid-beta (Abeta) or phosphorylated tau, are the characteristic feature of sporadic inclusion body myositis (s-IBM) muscle biopsies, we studied expression and immunolocalization of five ER chaperones, calnexin, ..
  12. ncbi Plasma cells in muscle in inclusion body myositis and polymyositis
    S A Greenberg
    Division of Neuromuscular Disease, Department of Neurology, Brigham and Women s Hospital, Harvard Medical School, Boston, MA, USA
    Neurology 65:1782-7. 2005
    Previous immunohistochemical studies of muscle from patients with inclusion body myositis and polymyositis found many more T cells than B cells, suggesting a role for intramuscular cell-mediated immune mechanisms rather than humoral ..
  13. ncbi The UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase gene is mutated in recessive hereditary inclusion body myopathy
    I Eisenberg
    Unit for Molecular Biology, Hadassah, Hospital, The Hebrew University Hadassah Medical School, Jerusalem, Israel
    Nat Genet 29:83-7. 2001
    ..Our findings indicate that GNE is the gene responsible for recessive HIBM...
  14. ncbi Steroid-responsive inclusion body myositis associated with endometrial cancer
    D T Alexandrescu
    Comprehensive Cancer Center, Our Lady of Mercy Medical Center, Bronx, New York 10466, USA
    Clin Exp Rheumatol 23:93-6. 2005
    b>Inclusion body myositis (IBM) is an uncommon chronic inflammatory myopathy. Although the association between other myopathies and cancer has been well established, the relationship between IBM and neoplasia is not completely understood...
  15. ncbi Histone H1 is released from myonuclei and present in rimmed vacuoles with DNA in inclusion body myositis
    Satoshi Nakano
    Department of Neurology, Kansai Medical University, Moriguchi 570 8507, Japan
    Neuromuscul Disord 18:27-33. 2008
    To investigate myonuclear alterations in sporadic inclusion body myositis (s-IBM), we immuno-localized histones in muscles in 11 patients. The examination showed that vacuolar rims were frequently positive for histone H1...
  16. ncbi Epidemiology of inclusion body myositis in the Netherlands: a nationwide study
    U A Badrising
    Department of Neurology, Leiden University Medical Center, The Netherlands
    Neurology 55:1385-7. 2000
    Epidemiologic data on inclusion body myositis (IBM) are scarce, and possibly biased, because they are derived from larger neuromuscular centers...
  17. ncbi 119th ENMC international workshop: trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10-12 October 2003, Naarden, The Netherlands
    Jessica E Hoogendijk
    Department of Neurology, University Medical Center, Heidelberg laan 100, Utrecht, CX 3584, The Netherlands
    Neuromuscul Disord 14:337-45. 2004
  18. pmc Hereditary inclusion body myopathy-linked p97/VCP mutations in the NH2 domain and the D1 ring modulate p97/VCP ATPase activity and D2 ring conformation
    Dalia Halawani
    The Nicholas Conor Institute for Pediatric Cancer Research, 9710 Scranton Road, Suite 170, San Diego, CA 92121, USA
    Mol Cell Biol 29:4484-94. 2009
    ..Therefore, we propose that hIBMPFTD p97/VCP mutants p97(R155P) and p97(A232E) possess structural defects that may compromise the mechanism of p97/VCP activity within large multiprotein complexes...
  19. pmc Theories of the pathogenesis of inclusion body myositis
    Steven A Greenberg
    Department of Neurology, Division of Neuromuscular Disease, Brigham and Women s Hospital, and Children s Hospital Informatics Program, Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA
    Curr Rheumatol Rep 12:221-8. 2010
    b>Inclusion body myositis is a progressive disease of the skeletal muscle. Here, specific theories of its pathogenesis are reviewed and general considerations pertaining to modeling of this disease discussed...
  20. pmc Interrelation of inflammation and APP in sIBM: IL-1 beta induces accumulation of beta-amyloid in skeletal muscle
    Jens Schmidt
    Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA
    Brain 131:1228-40. 2008
    ..degeneration, the two major hallmarks of the skeletal muscle pathology in sporadic inclusion body myositis (sIBM), have remained elusive...
  21. ncbi TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations
    Manuela Neumann
    Center for Neuropathology and Prion Research, Ludwig Maximilians University, Munich, Germany
    J Neuropathol Exp Neurol 66:152-7. 2007
    ..TDP-43 is a common pathologic substrate linking a variety of distinct patterns of FTLD-U pathology caused by different genetic alterations...
  22. ncbi Proinflammatory cell stress in sporadic inclusion body myositis muscle: overexpression of alphaB-crystallin is associated with amyloid precursor protein and accumulation of beta-amyloid
    I E Muth
    Department of Neurology and Department of Experimental and Clinical Neuroimmunology, University Medicine Gottingen, Waldweg 33, 37073 Gottingen, Germany
    J Neurol Neurosurg Psychiatry 80:1344-9. 2009
    In the pathology of sporadic inclusion body myositis (sIBM), the relevance of cell stress molecules such as the heat shock protein alphaB-crystallin, particularly in healthy appearing muscle fibres, has remained elusive.
  23. ncbi Sporadic inclusion body myositis: phenotypic variability and influence of HLA-DR3 in a cohort of 57 Australian cases
    M Needham
    Centre for Neuromuscular and Neurological Disorders, University of Western Australia, Australian Neuromuscular Research Institute ANRI, Queen Elizabeth II Medical Centre, Nedlands, Perth 6009, WA, Australia
    J Neurol Neurosurg Psychiatry 79:1056-60. 2008
    There have been few studies of the variability in the clinical phenotype in sporadic inclusion body myositis (sIBM) and it is not known whether the human leucocyte antigen (HLA) haplotype influences the phenotype and course of the ..
  24. ncbi Distal myopathy with rimmed vacuoles is allelic to hereditary inclusion body myopathy
    I Nishino
    Department of Neuromuscular Research, National Institute of Neuroscience, Kodaira, Tokyo, Japan
    Neurology 59:1689-93. 2002
    ..Recently, HIBM was shown to be associated with the mutations in the gene encoding the bifunctional enzyme, UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase (GNE)...
  25. ncbi Inflammation induces tau pathology in inclusion body myositis model via glycogen synthase kinase-3beta
    Masashi Kitazawa
    Department of Neurobiology and Behavior, University of California, Irvine, Irvine, CA 92697 4545, USA
    Ann Neurol 64:15-24. 2008
    b>Inclusion body myositis (IBM) is an inflammatory muscle disease, although the role of inflammation remains to be elucidated...
  26. ncbi Upregulation of thrombospondin-1(TSP-1) and its binding partners, CD36 and CD47, in sporadic inclusion body myositis
    Mohammad Salajegheh
    The Division of Neuromuscular Disease, Department of Neurology, Brigham and Women s Hospital, 75 Francis Street, Tower 5D, Boston, MA 02115, USA
    J Neuroimmunol 187:166-74. 2007
    ..The TSP-complex is another inflammatory mediator associated with chronic inflammation in IBM that may perpetuate the immune responses to local antigens in response to TNF-alpha...
  27. ncbi Apolipoprotein epsilon alleles in sporadic inclusion body myositis: a reappraisal
    Merrilee Needham
    Centre for Neuromuscular and Neurological Disorders, University of Western Australia, Queen Elizabeth II Medical Centre, Perth, Australia
    Neuromuscul Disord 18:150-2. 2008
    ..studies have differed as to whether APOE epsilon4 is a susceptibility factor for developing sporadic inclusion body myositis (sIBM), with a positive association being found only in an Australian cohort of cases...
  28. ncbi [Inclusion body myositis after interferon-alpha treatment in a patient with HCV and HTLV-1 infection]
    Yoko Warabi
    Department of Neurology, Tokyo Metropolitan Neurological Hospital
    Rinsho Shinkeigaku 44:609-14. 2004
    We report the first case of inclusion body myositis (IBM) which occurred after interferon-alpha treatment for chronic hepatitis C...
  29. ncbi Upregulated inducible co-stimulator (ICOS) and ICOS-ligand in inclusion body myositis muscle: significance for CD8+ T cell cytotoxicity
    Jens Schmidt
    Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Brain 127:1182-90. 2004
    ..Because in the muscle of patients with sporadic inclusion body myositis (sIBM) clonally expanded CD8+ T cells invade major histocompatibility complex (MHC) class I-expressing ..
  30. ncbi Expression of lysosome-related proteins and genes in the skeletal muscles of inclusion body myositis
    Toshihide Kumamoto
    Division of Neurology and Neuromuscular Disorders, Department of Immunology and Allergy, Oita Medical University, Idaigaoka 1 1, Hasama, 879 5593, Oita, Japan
    Acta Neuropathol 107:59-65. 2004
    Despite the unknown etiology and pathogenesis of sporadic inclusion body myositis (s-IBM), investigators have speculated that the lysosome system in muscle fiber plays a central role in rimmed vacuole formation, a hallmark of s-IBM...
  31. ncbi Sporadic inclusion body myositis in Japanese is associated with the MHC ancestral haplotype 52.1
    Adrian Phillip Scott
    School of Surgery and Pathology, M504, UWA, Stirling Highway, Nedlands, WA 6009, Perth WA, Australia
    Neuromuscul Disord 16:311-5. 2006
    In Caucasians, sporadic inclusion body myositis has been associated with the MHC ancestral haplotypes; HLA-A1, B8, DR3 (8.1AH) and HLA-B35, DR1 (35.2AH)...
  32. doi Interplay between inflammation and degeneration: using inclusion body myositis to study "neuroinflammation"
    Marinos C Dalakas
    Ann Neurol 64:1-3. 2008
  33. ncbi A prospective natural history study of inclusion body myositis: implications for clinical trials
    M R Rose
    Department of Neurology, King s Neurosciences Centre, King s College Hospital, London, UK
    Neurology 57:548-50. 2001
    Eleven patients with untreated inclusion body myositis (IBM) were prospectively studied during a 6-month period that included muscle strength, lean body mass, and muscle mass measurements...
  34. ncbi Inclusion body myositis functional rating scale: a reliable and valid measure of disease severity
    C E Jackson
    University of Texas Health Science Center, San Antonio, TX, USA
    Muscle Nerve 37:473-6. 2008
    We developed a disease-specific, 10-point functional rating scale for patients with inclusion body myositis (IBMFRS)...
  35. ncbi Sporadic inclusion body myositis: pathogenic considerations
    George Karpati
    Department of Neurology, McGill University, Montreal Neurological Institute, Montreal, Quebec, Canada
    Ann Neurol 65:7-11. 2009
    Sporadic inclusion body myositis is the commonest acquired disease of skeletal muscles after 50 years of age, and as such it has commanded a great deal of attention of investigators over the past 25 years...
  36. ncbi Anti-PM-Scl antibodies in a patient with inclusion body myositis
    A Selva-O'Callaghan
    Rheumatology (Oxford) 42:1016-8. 2003
  37. ncbi Improvement in aerobic capacity after an exercise program in sporadic inclusion body myositis
    Liam G Johnson
    Centre for Neuromuscular and Neurological Disorders, University of Western Australia, Perth, Western Australia
    J Clin Neuromuscul Dis 10:178-84. 2009
    The study aimed to investigate the effects of a combined functional and aerobic exercise program on aerobic capacity, muscle strength, and functional mobility in a group of patients with sporadic inclusion body myositis (IBM).
  38. ncbi Intravenous immunoglobulin for dysphagia of inclusion body myositis
    P Cherin
    Service de Medecine Interne I, Hopital Salpetriere, Paris, France
    Neurology 58:326. 2002
  39. ncbi Novel missense mutation and large deletion of GNE gene in autosomal-recessive inclusion-body myopathy
    Roberto Del Bo
    Dipartimento di Scienze Neurologiche, Centro Dino Ferrari, Universita degli Studi di Milano, IRCCS Ospedale Maggiore Policlinico, Via F Sforza 35, Milano 20122, Italy
    Muscle Nerve 28:113-7. 2003
    ..This is the first deletion event observed in a GNE allele and expands the molecular pathogenesis of autosomal-recessive h-IBM...
  40. ncbi Mycophenolate (CellCept) treatment of myasthenia gravis, chronic inflammatory polyneuropathy and inclusion body myositis
    N Mowzoon
    Department of Neurology, University of Miami School of Medicine (M712, PO Box 016960, Miami, FL 33101, USA
    J Neurol Sci 185:119-22. 2001
    ..demyelinating polyneuropathy (CIDP), one patient with secondary polymyositis (PM), and one patient with inclusion body myositis (IBM). Side effects were mild...
  41. pmc Sporadic inclusion body myositis: morphology, regeneration, and cytoskeletal structure of muscle fibres
    S Arnardottir
    Department of Clinical Neuroscience, Division of Neurology, Karolinska Hospital, Stockholm, Sweden
    J Neurol Neurosurg Psychiatry 75:917-20. 2004
    To characterise morphological abnormalities in relation to muscle fibre type in sporadic inclusion body myositis (s-IBM).
  42. ncbi Messenger RNA degradation may be inhibited in sporadic inclusion body myositis
    S Nakano
    Department of Neurology, Kansai Medical University, Moriguchi City, Japan
    Neurology 65:420-5. 2005
    To integrate an immune-mediated mechanism and the disturbed protein expression in sporadic inclusion body myositis (IBM).
  43. ncbi Difference in adhesion molecule expression (ICAM-1 and VCAM-1) in juvenile and adult dermatomyositis, polymyositis and inclusion body myositis
    Adriana M E Sallum
    Department of Pediatrics University of São Paulo Medical School, Brazil
    Autoimmun Rev 5:93-100. 2006
    ..In contrast, VCAM-1 seems not to play a major role in JDM, as previously described in PM, DM and IBM. Adhesion molecule expression in JDM presents a differential characteristic when compared to PM, DM and IBM...
  44. ncbi What determines quality of life in inclusion body myositis?
    R Sadjadi
    Department of Neurology, King s College Hospital and King s College London School of Medicine, University of London, London, UK
    J Neurol Neurosurg Psychiatry 81:1164-6. 2010
    ..a better choice from among the various ways we currently measure the severity of a muscle disease such as inclusion body myositis (IBM)...
  45. ncbi Macroautophagy as a pathomechanism in sporadic inclusion body myositis
    Jan D Lunemann
    Laboratory of Viral Immunobiology, Christopher H Browne Center for Immunology and Immune Diseases, The Rockefeller University, New York, New York 10021, USA
    Autophagy 3:384-6. 2007
    Skeletal muscle fibers show a high level of constitutive and starvation-induced macroautophagy. Sporadic Inclusion Body Myositis (sIBM) is the most common acquired skeletal muscle disease in patients above the age of 50 years and is ..
  46. ncbi Sporadic inclusion body myositis: HLA-DRB1 allele interactions influence disease risk and clinical phenotype
    Frank L Mastaglia
    Centre for Neuromuscular and Neurological Disorders, University of Western Australia, Australian Neuromuscular Research Institute ANRI, Queen Elizabeth II Medical Centre, Nedlands, Perth, WA 6009, Australia
    Neuromuscul Disord 19:763-5. 2009
    ..The findings indicate that interactions between the HLA-DRB1*03 allele and other alleles at the DRB1 locus can influence disease susceptibility and the clinical phenotype in sIBM...
  47. ncbi Amyloid-beta42 is preferentially accumulated in muscle fibers of patients with sporadic inclusion-body myositis
    Gaetano Vattemi
    USC Neuromuscular Center, Department of Neurology, University of Southern California Keck School of Medicine, Good Samaritan Hospital, Los Angeles, CA, 90017, USA
    Acta Neuropathol 117:569-74. 2009
    ..Thus, in s-IBM muscle fibers, Abeta42 is accumulated more than Abeta40. We suggest that Abeta42 oligomers and their cytotoxicity may play an important role in the s-IBM pathogenesis...
  48. ncbi Pathological consequences of VCP mutations on human striated muscle
    Christian U Hübbers
    Institute of Biochemistry I, University of Cologne, Cologne, Germany
    Brain 130:381-93. 2007
    ..The latter findings provide a novel link to VCP carbohydrate interactions in the complex pathology of IBMPFD...
  49. ncbi Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation
    Conrad C Weihl
    Department of Neurology, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, MO 63110, USA
    Hum Mol Genet 15:189-99. 2006
    ..Undegraded mutant DeltaF508-CFTR also accumulates in these aggregates. We conclude that IBMPFD mutations in p97/VCP disrupt ERAD and that this may contribute to the pathogenesis of IBMPFD...
  50. pmc Proteasome inhibition and aggresome formation in sporadic inclusion-body myositis and in amyloid-beta precursor protein-overexpressing cultured human muscle fibers
    Pietro Fratta
    Department of Neurology, USC Neuromuscular Center, Good Samaritan Hospital, Los Angeles, CA 90017 1912, USA
    Am J Pathol 167:517-26. 2005
    ..Accordingly, proteasome dysfunction in s-IBM muscle fibers may play a role in accumulation of misfolded, potentially cytotoxic proteins and may be induced by increased intracellular AbetaPP/Abeta...
  51. ncbi Mutations spectrum of GNE in hereditary inclusion body myopathy sparing the quadriceps
    Iris Eisenberg
    Molecular Biology Unit, Hadassah Hospital, The Hebrew University Hadassah Medical School, Jerusalem, Israel
    Hum Mutat 21:99. 2003
    ..The mechanism leading to this unique phenotype still remains to be elucidated...
  52. ncbi Distal myopathy with rimmed vacuoles: novel mutations in the GNE gene
    H Tomimitsu
    Department of Neurology and Neurological Science, Graduate School, Tokyo Medical and Dental University, 1 5 45 Yushima, Bunkyo ku, Tokyo 113 8519, Japan
    Neurology 59:451-4. 2002
    ..Seven out of nine patients had homozygous V572L mutation, one was a compound heterozygote with C303V and V572L mutations, and the remaining patient bore homozygous A631V mutation...
  53. ncbi Inclusion body myositis: review of recent literature
    Steven A Greenberg
    Department of Neurology, Brigham and Women s Hospital, 75 Francis Street, Boston, MA 02115, USA
    Curr Neurol Neurosci Rep 9:83-9. 2009
    b>Inclusion body myositis (IBM) is a progressive inflammatory skeletal muscle disease of unknown cause and without effective treatment. This article discusses existing literature, emphasizing disease mechanisms and models...
  54. ncbi Beta-amyloid peptide expression is sufficient for myotube death: implications for human inclusion body myopathy
    H W Querfurth
    Division of Neurology, St Elizabeth s Medical Center, Boston, MA 02135, USA
    Mol Cell Neurosci 17:793-810. 2001
    b>Inclusion body myositis (sIBM) is the most common disorder of skeletal muscle in aged humans...
  55. ncbi Novel GNE mutations in Italian families with autosomal recessive hereditary inclusion-body myopathy
    Aldobrando Broccolini
    Department of Neuroscience, Catholic University, Rome, Italy
    Hum Mutat 23:632. 2004
    ..Interestingly, in two of our families distinct mutations affected nucleotide c.616 in exon 3 (c.616delG and c.616G>A). The possibility of specific portions of the gene being more prone to mutations remains to be elucidated...
  56. ncbi Normal sialylation of serum N-linked and O-GalNAc-linked glycans in hereditary inclusion-body myopathy
    Paul J M Savelkoul
    Mol Genet Metab 88:389-90. 2006
  57. ncbi Inflammatory disorders of muscle: progress in polymyositis, dermatomyositis and inclusion body myositis
    Marinos C Dalakas
    Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892 1382, USA
    Curr Opin Neurol 17:561-7. 2004
    ..To provide an update on the major advances in inflammatory myopathies...
  58. ncbi A pilot randomized trial of oxandrolone in inclusion body myositis
    S B Rutkove
    Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA
    Neurology 58:1081-7. 2002
    b>Inclusion body myositis (IBM) remains without effective therapy. As anabolic steroids have myotrophic properties, the authors studied whether a synthetic androgen, oxandrolone, would have efficacy in IBM.
  59. pmc Casein kinase 1 alpha associates with the tau-bearing lesions of inclusion body myositis
    Theresa J Kannanayakal
    Center for Molecular Neurobiology, Department of Molecular and Cellular Biochemistry, College of Medicine, The Ohio State University, 1060 Carmack Road, Columbus, OH 43210, United States
    Neurosci Lett 431:141-5. 2008
    b>Inclusion body myositis and Alzheimer's disease are age-related disorders characterized in part by the appearance of intracellular lesions composed of filamentous aggregates of the microtubule-associated protein tau...
  60. pmc Fast-twitch sarcomeric and glycolytic enzyme protein loss in inclusion body myositis
    Kenneth C Parker
    Harvard Partners Center for Genetics and Genomics, Proteomics Core, Harvard Medical School, Boston, Massachusetts USA
    Muscle Nerve 39:739-53. 2009
    b>Inclusion body myositis (IBM) is an inflammatory disease of skeletal muscle of unknown cause...
  61. ncbi Sporadic inclusion body myositis correlates with increased expression and cross-linking by transglutaminases 1 and 2
    Y C Choi
    Department of Neurology, College of Medicine, Yonsei University, Seoul 135 270, Republic of Korea
    J Biol Chem 275:8703-10. 2000
    Sporadic inclusion body myositis (SIBM) is characterized by vacuolar degeneration of muscle fibers and intrafiber clusters of paired helical filaments with abnormal amyloid deposition...
  62. ncbi Beta-amyloid is a substrate of autophagy in sporadic inclusion body myositis
    Jan D Lunemann
    Laboratory of Viral Immunobiology, Christopher H Browne Center for Immunology and Immune Diseases, The Rockefeller University, New York, NY 10021, USA
    Ann Neurol 61:476-83. 2007
    Sporadic Inclusion Body Myositis (sIBM) is the most common acquired muscle disease in patients above 50 years of age...
  63. pmc Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia
    Conrad C Weihl
    Department of Neurology, Washington University School of Medicine, Saint Louis, MO 63110, USA
    Neuromuscul Disord 19:308-15. 2009
    ....
  64. ncbi Associations with autoimmune disorders and HLA class I and II antigens in inclusion body myositis
    U A Badrising
    Department of Neurology, K5Q, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands
    Neurology 63:2396-8. 2004
    Whether autoimmune mechanisms play a role in the pathogenesis of inclusion body myositis (IBM) is unknown...
  65. ncbi Congenital myopathy with abundant ring fibres, rimmed vacuoles and inclusion body myositis-type inclusions
    A Fidzianska
    Neuromuscular Unit, Medical Research Centre, Polish Academy of Sciences, Warsaw, Poland
    Neuropediatrics 34:40-4. 2003
    ..ring fibres and numerous rimmed vacuoles as well as intracytoplasmic and intranuclear inclusions of the inclusion body myositis-type...
  66. ncbi Expression of IFN-gamma-inducible chemokines in inclusion body myositis
    Raghavanpillai Raju
    National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 1382, USA
    J Neuroimmunol 141:125-31. 2003
    ..of effector T cells, we examined their expression in the muscle biopsies of patients with sporadic inclusion body myositis (s-IBM) and disease controls...
  67. ncbi Rimmed vacuoles and the added value of SMI-31 staining in diagnosing sporadic inclusion body myositis
    M F van der Meulen
    Department of Neurology, G 03 228, Division of Neuromuscular Disorders, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX, The, Utrecht, Netherlands
    Neuromuscul Disord 11:447-51. 2001
    Problems in diagnosing sporadic inclusion body myositis may arise if all clinical features fit a diagnosis of polymyositis, but the muscle biopsy shows some rimmed vacuoles...
  68. pmc Sporadic inclusion body myositis: variability in prevalence and phenotype and influence of the MHC
    F L Mastaglia
    Centre for Neuromuscular and Neurological Disorders, University of Western Australia
    Acta Myol 28:66-71. 2009
    Sporadic inclusion body myositis (sIBM) is the most common myopathy presenting over the age of 40 years but its prevalence varies considerably in different populations...
  69. ncbi Inclusion body myositis: old and new concepts
    A A Amato
    Department of Neurology, Brigham and Women s Hospital, Harvard Medical School, 75 Francis St, Boston, MA 02115, USA
    J Neurol Neurosurg Psychiatry 80:1186-93. 2009
    b>Inclusion body myositis (IBM) is the most common idiopathic inflammatory myopathy occurring in patients over the age of 50 years and probably accounts for about 30% of all inflammatory myopathies...
  70. ncbi Gene expression profile in the muscles of patients with inflammatory myopathies: effect of therapy with IVIg and biological validation of clinically relevant genes
    Raghavan Raju
    Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10 Room 4N252, 10 Center Drive, Bethesda, MD 20892, USA
    Brain 128:1887-96. 2005
    ..obtained before and after therapy from patients with dermatomyositis (DM) who improved and patients with inclusion body myositis (sIBM) who did not improve after controlled trials with three monthly intravenous immunoglobulin (IVIg) ..
  71. ncbi Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice
    Conrad C Weihl
    Department of Neurology, Washington University School of Medicine, 660 S Euclid Avenue, Saint Louis, MO 63110, USA
    Hum Mol Genet 16:919-28. 2007
    ..TgVCP-RH animals will be a valuable tool for understanding the pathogenesis of IBM and the role of the UPS in skeletal muscle...
  72. ncbi CCR7+ myeloid dendritic cells together with CCR7+ T cells and CCR7+ macrophages invade CCL19+ nonnecrotic muscle fibers in inclusion body myositis
    Maki Tateyama
    Department of Neurology, Tohoku University School of Medicine, Sendai, Japan
    J Neurol Sci 279:47-52. 2009
    ..investigated the expression of this chemokine system in the muscles of seven patients with inclusion body myositis (IBM)...
  73. ncbi Proteomic analysis of inclusion body myositis
    Jie Li
    Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke NINDS, National Institutes of Health NIH, Bethesda, Maryland, USA
    J Neuropathol Exp Neurol 65:826-33. 2006
    Sporadic inclusion body myositis (IBM) is the most frequently acquired inflammatory myopathy of late adult life, yet its diagnostic criteria and pathogenesis remain poorly defined...
  74. ncbi Four novel mutations associated with autosomal recessive inclusion body myopathy (MIM: 600737)
    D Darvish
    HIBM Research Group, 16661 Ventura Blvd, 311, Encino, CA 91436, USA
    Mol Genet Metab 77:252-6. 2002
    ....
  75. ncbi Inclusion-body myositis, a multifactorial muscle disease associated with aging: current concepts of pathogenesis
    Valerie Askanas
    USC Neuromuscular Center, Department of Neurology, University of Southern California Keck School of Medicine, Good Samaritan Hospital, Los Angeles, California 90017 1912, USA
    Curr Opin Rheumatol 19:550-9. 2007
    ..About 100 papers related to the subject were published in 2006 and the first part of 2007 (we cite only articles most relevant to this review)...
  76. ncbi Tau aggregates are abnormally phosphorylated in inclusion body myositis and have an immunoelectrophoretic profile distinct from other tauopathies
    C A Maurage
    INSERM U422, Faculte de Medecine, 1 place de Verdun, Lille Cedex, France
    Neuropathol Appl Neurobiol 30:624-34. 2004
    Sporadic inclusion body myositis (s-IBM) is the most frequent progressive acquired inflammatory myopathy in people older than 50 years...
  77. ncbi A controlled study of intravenous immunoglobulin combined with prednisone in the treatment of IBM
    M C Dalakas
    Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA
    Neurology 56:323-7. 2001
    ..with prednisone improves muscle strength and alters endomysial inflammation in patients with sporadic inclusion body myositis (s-IBM)...
  78. ncbi Preservation of in vitro muscle fiber function in dermatomyositis and inclusion body myositis: a single fiber study
    Lisa S Krivickas
    Department of Physical Medicine and Rehabilitation, Spaulding Rehabilitation Hospital and Harvard Medical School, 125 Nashua St, Boston, MA 02114, USA
    Neuromuscul Disord 15:349-54. 2005
    Five patients with untreated dermatomyositis, five with inclusion body myositis, and 16 healthy elderly volunteer subjects (controls) underwent open (dermatomyositis and inclusion body myositis) or percutaneous (controls) muscle biopsy...
  79. pmc Familial inflammatory inclusion body myositis
    B Ranque-Francois
    Internal Medicine Department, La Pitie Salpetriere Hospital, 75651 Paris, France
    Ann Rheum Dis 64:634-7. 2005
    To compare familial inflammatory inclusion body myositis (IBM) with hereditary inclusion body myopathies and sporadic IBM.
  80. ncbi Report of a patient with inclusion body myositis and CD8+ chronic lymphocytic leukaemia--post-mortem analysis of muscle and brain
    S Arnardottir
    Department of Clinical Neuroscience, Karolinska Hospital, Karolinska Institutet, Stockholm, Sweden
    Acta Neurol Scand 103:131-5. 2001
    We report a 73-year-old woman with sporadic inclusion body myositis (s-IBM) and a T-cell chronic lymphocytic leukaemia (T-CLL)...
  81. ncbi Inclusion body myositis. Clinical features and clinical course of the disease in 64 patients
    Umesh A Badrising
    Dept of Neurology, Leiden University Medical Centre, Leiden, The Netherlands
    J Neurol 252:1448-54. 2005
    The clinical features of inclusion body myositis (IBM) were of minor importance in the design of consensus diagnostic criteria, mainly because of controversial views on the specificity of signs and symptoms, although some authors ..
  82. ncbi Inclusion body myositis associated with hepatitis C virus infection
    Y Tsuruta
    Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan
    Fukuoka Igaku Zasshi 92:370-6. 2001
    b>Inclusion body myositis (IBM) is a chronic progressive inflammatory myopathy in elders. Three patients with chronic hepatitis C developed IBM...
  83. ncbi Correlation of muscle biopsy, clinical course, and outcome in PM and sporadic IBM
    Nizar Chahin
    Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
    Neurology 70:418-24. 2008
    To correlate muscle biopsy findings with prebiopsy and postbiopsy clinical course and response to therapy in polymyositis (PM) and sporadic inclusion body myositis (IBM).
  84. ncbi Sporadic inclusion body myositis: a continuing puzzle
    M Needham
    Centre for Neuromuscular and Neurological Disorders, Level 4, A Block, Australian Neuromuscular Research Institute, Queen Elizabeth II Medical Centre, University of Western Australia, Perth, WA 6009, Australia
    Neuromuscul Disord 18:6-16. 2008
    There is now compelling evidence that sporadic inclusion body myositis (sIBM) is a muscle-specific autoimmune disease in which both T and B-cells play a part and in which both cytotoxic muscle fibre necrosis and degeneration occur...
  85. ncbi Patterns of muscle involvement in inclusion body myositis: clinical and magnetic resonance imaging study
    B A Phillips
    Centre for Neuromuscular and Neurological Disorders, Australian Neuromuscular Research Institute, University of Western Australia, Perth, Western Australia, Australia
    Muscle Nerve 24:1526-34. 2001
    The differential patterns of muscle involvement in the upper and lower limbs in sporadic inclusion body myositis (sIBM) were examined in 18 patients using both quantitative and manual muscle testing as well as magnetic resonance imaging (..
  86. ncbi Inclusion body myositis: genetic factors, aberrant protein expression, and autoimmunity
    A Oldfors
    Göteborg Neuromuscular Center, Department of Pathology, Sahlgrenska University Hospital, Goteborg, Sweden
    Curr Opin Rheumatol 13:469-75. 2001
    Sporadic inclusion body myositis (s-IBM) is an inflammatory myopathy mainly affecting elderly individuals. It has a chronic progressive course leading to severe disability. Immunosuppressive treatment is in most instances ineffective...
  87. ncbi High-dose vitamin C therapy for inclusion body myositis
    T Yamada
    Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812 8582, Japan
    Fukuoka Igaku Zasshi 92:99-104. 2001
    The efficiency of high-dose vitamin C therapy for inclusion body myositis (IBM) was assessed.
  88. ncbi Macrophagic myofasciitis associated with inclusion body myositis: a report of three cases
    P Cherin
    Médecine Interne I, CHU Pitie Salpetriere, 47 Boulevard de l Hopital, 75013, Paris, France
    Neuromuscul Disord 11:452-7. 2001
    We describe three patients with macrophagic myofasciitis and inclusion body myositis. All patients fulfilled diagnostic criteria for inclusion body myositis and myopathologic criteria for macrophagic myofasciitis...
  89. pmc TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia
    C C Weihl
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Neurol Neurosurg Psychiatry 79:1186-9. 2008
    ..TDP-43 inclusions were also found in 78% of sporadic inclusion body myositis (sIBM) muscles...
  90. ncbi Shared blood and muscle CD8+ T-cell expansions in inclusion body myositis
    Dalia Dimitri
    service de médecine interne 1, Hopital Pitie Salpetriere, Paris, France
    Brain 129:986-95. 2006
    b>Inclusion body myositis (IBM) is the most frequent inflammatory myopathy over the age of fifty...
  91. ncbi Inclusion-body myositis: clinical, diagnostic, and pathologic aspects
    W King Engel
    The Neuromuscular Center, Department of Neurology, University of Southern California Keck School of Medicine, Good Samaritan Hospital, Los Angeles, CA, USA
    Neurology 66:S20-9. 2006
    ..Available treatments are of only slight, temporary benefit for only some s-IBM patients, indicating a desperate need for definitive therapies...
  92. ncbi Inclusion body myositis: an underdiagnosed myopathy of older people
    Sunil K Munshi
    Department of Medicine for the Elderly, Leicester Royal Infirmary, Infirmary Square, Leicester LE1 5WW, UK
    Age Ageing 35:91-4. 2006
    b>Inclusion body myositis (IBM), a condition characterised by progressive muscle weakness and inclusion bodies visible on muscle biopsy, is the most common type of myopathy in patients over 50 years of age...
  93. ncbi Dysphagia in inclusion body myositis: clinical features, management, and clinical outcome
    Terry H Oh
    Department of Physical Medicine and Rehabilitation, Mayo Clinic, Rochester, Minnesota 55905, USA
    Am J Phys Med Rehabil 87:883-9. 2008
    To evaluate the clinical features, treatment strategies, and outcome of dysphagia in patients with inclusion body myositis.
  94. ncbi Inclusion body myositis: new insights into pathogenesis
    Michael J Garlepp
    School of Pharmacy, Curtin University of Technology, Australia
    Curr Opin Rheumatol 20:662-8. 2008
    The pathogenesis of sporadic inclusion body myositis is complex and the disease has a relentless course. Recent observations regarding possible mechanisms of disease may provide targets for therapy.
  95. ncbi Myostatin is increased and complexes with amyloid-beta within sporadic inclusion-body myositis muscle fibers
    Sławomir Wójcik
    USC Neuromuscular Center, Department of Neurology, University of Southern California Keck School of Medicine, Good Samaritan Hospital, 637 S Lucas Ave, Los Angeles, CA, 90017 1912, USA
    Acta Neuropathol 110:173-7. 2005
    ..Our study suggests that myostatin/myostatin precursor, either alone, or bound to Abeta, may play a novel role in the pathogenesis of s-IBM...
  96. ncbi Pathogenic accumulation of APP in fast twitch muscle of IBM patients and a transgenic model
    Michael C Sugarman
    Department of Neurobiology and Behavior, University of California, 1109 Gillespie Neuroscience Facility, Irvine, CA 92697 4545, USA
    Neurobiol Aging 27:423-32. 2006
    b>Inclusion body myositis (IBM) is the most common age-related degenerative skeletal muscle disorder. The aberrant intracellular accumulation of the beta-amyloid (Abeta) peptide within skeletal muscle is a pathological hallmark of IBM...
  97. ncbi T cell receptor profiling in muscle and blood lymphocytes in sporadic inclusion body myositis
    M Salajegheh
    Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Neurology 69:1672-9. 2007
    ..Sporadic IBM (sIBM) is characterized by invasion of non-necrotic MHC-I class-expressing muscle fibers by clonally expanded CD8+ cells. Whether the endomysial cells expand in situ or are recruited from the circulation is unclear...
  98. ncbi Polymyositis and dermatomyositis
    Marinos C Dalakas
    Neuromuscular Diseases Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 1382, USA
    Lancet 362:971-82. 2003
    ..Early initiation of therapy is essential, since both polymyositis and dermatomyositis respond to immunotherapeutic agents. New immunomodulatory agents currently being tested in controlled trials may prove promising for difficult cases...
  99. ncbi Sporadic inclusion body myositis: pilot study on the effects of a home exercise program on muscle function, histopathology and inflammatory reaction
    Snjolaug Arnardottir
    Department of Clinical Neuroscience Division of Neurology, Karolinska Hospital, SE 171 76 Stockholm, Sweden
    J Rehabil Med 35:31-5. 2003
    To evaluate the safety and effect of a home training program on muscle function in 7 patients with sporadic inclusion body myositis.
  100. ncbi Anti-T-lymphocyte globulin treatment in inclusion body myositis: a randomized pilot study
    C Lindberg
    Department of Neurology, Sahlgrenska University Hospital Molndal, Sahlgrenska NeuroMuscular Center, Gothenburg, Sweden
    Neurology 61:260-2. 2003
    The authors performed an open, randomized trial in patients with inclusion body myositis comparing 1) 12-month treatment with oral methotrexate 7...
  101. ncbi Resistance training with vascular occlusion in inclusion body myositis: a case study
    Bruno Gualano
    School of Physical Education and Sport, University of Sao Paulo, Sao Paulo, Brazil
    Med Sci Sports Exerc 42:250-4. 2010
    b>Inclusion body myositis (IBM) is a rare idiopathic inflammatory myopathy that produces remarkable muscle weakness...

Research Grants62

  1. Acquisition of a TIRF/Widefield fluorescence microscope for cell biology and neur
    Bryon D Grove; Fiscal Year: 2013
    ..the University of North Dakota including Alzheimers[unreadable] disease, Parkinson[unreadable]s disease, inclusion body myositis (IBM), epilepsy and many more...
  2. Role of Presenilin in Idiopathic Dilated Cardiomyopathy
    Federica Del Monte; Fiscal Year: 2013
    ..diseases such as primary systemic amyloidosis, diabetes, cystic fibrosis, neurodegenerative diseases and inclusion body myositis. Although the molecular mechanisms by which these pathologies develop might be different, they are ..
  3. Post-9/11 Incidence of Systemic Autoimmune Diseases in the FDNY Cohort
    Mayris P Webber; Fiscal Year: 2013
    ..incidence of confirmed cases of SAID in the FDNY population from 9/11/2001 until 9/10/2013; and Aim 2: Perform a nested case-control study to estimate the effect size of WTC exposure on confirmed cases of all subtypes of SAID combined ..
  4. Multiparametric Classification of Muscle Damage in Inflammatory Myopathy
    Jane Park; Fiscal Year: 2013
    ..The idiopathic inflammatory myopathies (IIM), including dermatomyositis (DM), polymyositis (PM), and inclusion body myositis (IBM), are autoimmune diseases resulting in muscle inflammation, weakness, and pain...
  5. Discovery of Novel Autoantigens in Patients with Inclusion Body Myositis
    KEVIN C O'CONNOR; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Inclusion body myositis (IBM) is one of a group of muscle diseases known as the inflammatory myopathies, which are characterized by chronic muscle inflammation accompanied by muscle weakness...
  6. Role of RyRs, DHPRs and Ca2+ Entry in the Inclusion Body Myositis
    Alexander Shtifman; Fiscal Year: 2010
    ..proteins on Ca2+ dysregulation in skeletal muscle as it relates to a skeletal muscle disorder, the Inclusion Body Myositis (IBM). Dr...
  7. Characterization of Autoreactivity to Muscle Proteins in Inclusion Body Myositis
    Mohammad Salajegheh; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Inclusion body myositis (IBM) is a common inflammatory myopathy of the elderly...
  8. Screening for drug targets in a Drosophila model of muscle degeneration
    EDWARD KWAME OWUSU ANSAH; Fiscal Year: 2010
    ....
  9. A Multidisciplinary Analysis of Gelsolin Amyloid Disease
    Jeffery W Kelly; Fiscal Year: 2010
    ..recapitulates many features of FAF pathology, including the intracellular inclusions also associated with inclusion body myositis (IBM), the most common muscle degenerative disease in the aging population...
  10. Idiopathic Inflammatory Myopathies: Improving Diagnosis and Predicting Outcomes
    Lisa Christopher Stine; Fiscal Year: 2010
    ..Allan Gelber, Antony Rosen, and Paul Plotz. IIM, including dermatomyositis, polymyositis, and inclusion body myositis, constitute the largest subset of acquired myopathies and often affect adults in their prime...
  11. Sialic acid has putative signaling roles in muscle disorders of aging
    Nam Pham; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Sporadic inclusion body myositis (sIBM) is the most common acquired muscle disease associated with aging. sIBM afflicts individuals older than 50 years of age...
  12. TRANSGENIC MOUSE MODEL OF INCLUSION BODY MYOSITIS
    Frank LaFerla; Fiscal Year: 2001
    The overall long-term objective of this FIRST proposal is to understand the molecular pathogenesis of inclusion body myositis (IBM), the most common muscle disease in personals over 50 years of age...
  13. MITOCHONDRIAL DNA DEPLETION IN HUMAN DISEASE
    Tuan Vu; Fiscal Year: 2000
    ..I am eager to undertake this study under the sponsorship of Dr. DiMauro and in collaboration with other members of the H. Houston Merritt Clinical Research Center. ..
  14. Beta-Amyloid & Cell Death Mechanisms in Skeletal Muscle
    Henry Querfurth; Fiscal Year: 2005
    DESCRIPTION (Adapted from applicant's abstract): Inclusion Body Myositis (IBM) is the most common muscle disorder in patients over 50 years of age...
  15. Alzheimer Vaccines: Noninvasive Vaccination by DNA-Base*
    Ken Ichiro Fukuchi; Fiscal Year: 2005
    ..The nasal membrane application procedure eliminates pain and other problems associated with needle injection, protein purification, and adjuvant modification, and so may reduce medical costs. ..
  16. Modulating IBM pathology in transgenic mice
    Frank LaFerla; Fiscal Year: 2005
    ..proposes to develop and characterize novel transgenic mouse models to study the molecular pathogenesis of inclusion body myositis (IBM)...
  17. Skin-patch Vaccination against Alzheimer's Disease
    Ken Ichiro Fukuchi; Fiscal Year: 2002
    Alzheimer's disease (AD) and inclusion body myositis (IBM) share a number of common pathologies such as deposits of amyloid beta-protein (AB) and paired helical filaments, although such pathologies are mostly restricted to brain for AD ..
  18. ECTOPIC EXPRESSION OF THE PRION PROTEIN
    Patrick Bosque; Fiscal Year: 1999
    ..in these animals may also demonstrate similarities between the mechanism of prion disease and inclusion body myositis or type Il diabetes mellitus. If so, these animals may serve as models of these conditions...
  19. Proteomic Studies in Inclusion Body Myositis
    Steven Greenberg; Fiscal Year: 2009
    DESCRIPTION (provided by applicant): Inclusion body myositis (IBM) is a disease of unknown cause characterized by slowly progressive painless weakness and with no effective treatment...
  20. AAA ATPase p97/VCP and Inclusion Body Myopathy
    CONRAD WEIHL; Fiscal Year: 2009
    ..goal to investigate the cellular mechanisms of aging in relation to skeletal muscle disorders, using inclusion body myositis (IBM) as a prototypical disease...
  21. Role of RyRs and DHPRs and Ca2+ Entry in beta-amyloid mediated Ca2+ dysregulation
    Alexander Shtifman; Fiscal Year: 2009
    ..clinical scientist with extensive experience in adenoviral gene delivery and in the field of the Inclusion Body Myositis (IBM) and Dr...
  22. Gene Expression in Inflammatory Myopathies
    Steven Greenberg; Fiscal Year: 2005
    ..This work may provide further diagnostic approaches to these disorders and contribute to the understanding of their pathogenesis. ..
  23. Pathogenic Role of Abeta, tau and Inflammation in Inclusion Body Myositis
    Masashi Kitazawa; Fiscal Year: 2008
    unreadable] DESCRIPTION (provided by applicant): Inclusion body myositis (IBM) is the leading age-related skeletal muscle disorder, yet its etiology remains unknown, nor do effective treatments exist...
  24. A Pilot Study of Etanercept in Dermatomyositis
    Anthony Amato; Fiscal Year: 2007
    ..The information gained from this study is necessary in order to design larger therapeutic trials of etanercept and other agents in DM. ..
  25. INCLUSION BODY MYOSITIS: PILOT STUDY OF B-INTERFERON 1A
    Rabi Tawil; Fiscal Year: 2002
    b>Inclusion body myositis (IBM), is the most common acquired disease of muscle in adults over the age of 50. IBM has a typical clinical presentation but requires muscle biopsy for definitive diagnosis...
  26. Genetic Risks for Medication-Related Hemorrhagic Stroke
    Steven Greenberg; Fiscal Year: 2007
    ..This proposal is thus likely to form the foundation of a powerful, open-ended search for a panel of genetic tests to determine an individual's risk for Warfarin-related ICH. ..
  27. MECHANISM OF PAIN IN PATIENTS WITH FIBROMYALGIA SYNDROME
    Roland Staud; Fiscal Year: 2008
    ..In addition, our findings may contribute to the understanding of pain mechanisms related to other chronic pain disorders. ..
  28. IGF-I/ALS Trial
    Eric Sorenson; Fiscal Year: 2007
    ..The preliminary data from our prior trial allows design of a phase III treatment trial that will yield a definitive result about the treatment effect of IGF-I. ..
  29. Dichlorphenamide vs Acetazolamide for Periodic Paralysis
    Robert C Griggs; Fiscal Year: 2010
    ..abstract_text> ..
  30. BIOLOGY OF HUMAN INTRAEPITHELIAL LYMPHOCYTES
    Ellen Ebert; Fiscal Year: 2002
    ..These studies will determine the mechanism of IEL chemotaxis toward secreted products of ECs, why IL-15 is more potent than IL-2 in inducing LAK activity by IELs, and how IL-10 and IL-12 augment this activity. ..
  31. Immune cell dynamics during central nervous system viral infection
    JUAN DE LA TORRE; Fiscal Year: 2008
    ..abstract_text> ..
  32. Peripheral and Central Mechanism of Pain in Patients with Fibromyalgia
    ROLAND M STAUD; Fiscal Year: 2010
    ..Either way, we will provide evidence that will characterize the role of local anesthesia, placebo analgesia, or both. Thus patients with FM and other similar pain syndromes may strongly benefit from the results of our study. ..
  33. Alzheimer disease biomarkers in lens
    Peter H Frederikse; Fiscal Year: 2010
    ..These studies build on our earlier studies to understand the role of AbetaPP and Abeta in the lens which are already providing a new and specific conceptual basis for understanding cataract and its links with the brain. ..
  34. 6th International Symposium on FAP Disorders and 5th...
    Joel Buxbaum; Fiscal Year: 2006
    ..abstract_text> ..
  35. Nervous System Channelopathies: Pathogenesis & Treatment
    ROBERT GRIGGS; Fiscal Year: 2005
    ..They may also offer a window for understanding common disorders likely to be caused by CNS channel mutations/dysfunction such as migraine and epilepsy. ..
  36. Mechanisms of Tau-Based Neurodegeneration
    Mark Forman; Fiscal Year: 2005
    ..This proposal will also facilitate my transition from a trainee to a fully independent experimental neuropathologist. ..
  37. IN VIVO TREATMENT OF EAE WITH ANTI I-A ANTIBODIES
    Lawrence Steinman; Fiscal Year: 2001
    ..Aim 5 is to assess the possibility of using DNA vaccination against TCR Vb for treatment of EAE. It is hoped that these studies will provide the basis for future clinical trials in MS patients. ..
  38. T cell Interactions in the CNS During Viral Persistence
    Dorian McGavern; Fiscal Year: 2005
    ..The main objective of this research is to define the immunologic parameters that contribute to viral clearance versus persistence in the CNS and foster approaches that tip the balance in favor of clearance. ..
  39. NC-758 for Prevention of Recurrent Cerebral Hemorrhage
    Steven Greenberg; Fiscal Year: 2005
    ..In addition to testing a promising agent for CAA, the proposed study will generate the organization and pilot data to serve as a springboard for future trials of emerging anti-amyloid treatments. ..
  40. QUANTITATIVE FUNCTION IN MOTOR NEURON DISEASE
    Eric Sorenson; Fiscal Year: 2004
    ..This will become increasingly important as our population ages, increasing demands for medical and social resources. ..
  41. Intramolecular Regulation of Ryanodine Receptors
    Alexander Shtifman; Fiscal Year: 2004
    ..This work will provide novel information needed to better understand the process of EC coupling and also may shed new light on the nature of muscle disorders such as malignant hyperthermia. ..
  42. In Situ Analysis of Hepatitis B virus-specific T cells
    Dorian McGavern; Fiscal Year: 2006
    ..The main objective of this research is to more precisely define the contribution of HBV-specific CTL to viral clearance and pathogenesis by providing the first comprehensive analysis of these cells in situ. ..
  43. ApoJ (Clusterin) in Alzheimer's Disease and Aging
    Caleb Finch; Fiscal Year: 2006
    ..In vitro studies with primary glial cultures from wildtype and apoJ-deficient mice will examine mechanisms of apoJ in microglial activation and in the sensitivity of neurons to AB. ..
  44. Novel treatment for muscle disease: Fueling the pipeline and finding the product
    ROBERT GRIGGS; Fiscal Year: 2006
    ..unreadable] [unreadable] [unreadable]..
  45. CRP, inflammation, and neurodegeneration during aging
    Caleb Finch; Fiscal Year: 2006
    ..F1 hybrids will be made with APPswe/ind and the existing systemic huCRPtg (Y1) and with the neuron-specific huCRPtg (when available). The F1 hybrids will be examined for changes in deposits of Abeta-amyloid. ..
  46. Course Development in Neurobiology of Disease at the University of Iowa
    Daniel Tranel; Fiscal Year: 2006
    ..More than 35 neuroscience faculty at Iowa, experts in a wide range of diseases, have expressed a strong interest in participating in this course. [unreadable] [unreadable]..
  47. Plan forTrial to find Optimum Steroid Regimen in Duchenne Muscular Dystrophy
    ROBERT GRIGGS; Fiscal Year: 2006
    ....
  48. SYMPOSIUM ON ORGANISMS WITH SLOW AGING
    Caleb Finch; Fiscal Year: 2002
    ..A volunteer poster session will expand the range of organisms on the formal program and support the development of further interests by students and by researchers not so far identified with this new area. ..
  49. ALZHEIMER PATHOPHYSIOLOGY IN HUMAN CATARACT FORMATION
    PETER FREDERIKSE; Fiscal Year: 2002
    ..abstract_text> ..
  50. Large Scale Images of Gene Transcription in MS and EAE
    Lawrence Steinman; Fiscal Year: 2004
    ..The discovery of the role of osteopontin, first identified in large scale profiles, exemplifies how this approach may help us to understand MS and to develop new therapies. ..
  51. ADAPT 78 IN OXIDANT STRESS, AGING AND NEURODEGENERATION
    Kelvin Davies; Fiscal Year: 2004
    ..abstract_text> ..
  52. Novel Designs and Outcome Measures for Bench to Bedside Research on NMD
    ROBERT GRIGGS; Fiscal Year: 2007
    ..In this effort to provide new treatments for neuromuscular disease, this conference will help to further public health goals. [unreadable] [unreadable] [unreadable]..
  53. 'Training in Endocrinology and Neurobiology of Aging'
    Caleb Finch; Fiscal Year: 2007
    ..Opportunities for human studies on aging, including ethnic differences in aging processes, are provided through the Alzheimer Disease Research Center and the Diabetes Research Center. ..
  54. Pathogenesis and treatment of the periodic paralyses
    ROBERT GRIGGS; Fiscal Year: 2004
    ....
  55. Phase II Therapeutic Trial of Mexiletine
    Richard J Barohn; Fiscal Year: 2010
    ..These secondary measures will be quantified by lndividual Neuromuscular Quality of Life (INQOL), SF-36 (adults), SF-10 (children), grip myotonia, physical exam, and electrophysiological testing, respectively. ..
  56. Experimental Therapeutics of Neuromuscular Disease
    ROBERT GRIGGS; Fiscal Year: 2008
    ..unreadable] [unreadable] [unreadable] [unreadable]..
  57. TRANSTHYRETIN DEPOSITION IN AGING TRANSGENIC MICE
    Joel Buxbaum; Fiscal Year: 2004
    ..Investigation of the model may allow us to identify and study such mechanisms which, in turn, may provide insight into aging and offer potential therapeutic targets in more than one form of human amyloidosis. ..