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 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
  2. 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
  3. 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
  4. 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
  5. 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
  6. 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
  7. doi 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
  8. doi 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
  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 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

Detail Information

Publications221 found, 100 shown here

  1. 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 ..
  2. 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)...
  3. 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.
  4. 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...
  5. 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, ..
  6. 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...
  7. doi 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...
  8. doi 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.
  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 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...
  11. 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...
  12. 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...
  13. 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...
  14. 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
    ....
  15. 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...
  16. doi 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).
  17. 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...
  18. ncbi Anti-PM-Scl antibodies in a patient with inclusion body myositis
    A Selva-O'Callaghan
    Rheumatology (Oxford) 42:1016-8. 2003
  19. ncbi Intravenous immunoglobulin for dysphagia of inclusion body myositis
    P Cherin
    Service de Medecine Interne I, Hopital Salpetriere, Paris, France
    Neurology 58:326. 2002
  20. 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...
  21. 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...
  22. 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).
  23. 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).
  24. 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...
  25. 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...
  26. doi 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...
  27. 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 ..
  28. doi 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 ..
  29. doi 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)...
  30. doi 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...
  31. 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
  32. 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...
  33. 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...
  34. 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...
  35. 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...
  36. doi 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...
  37. 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...
  38. 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...
  39. 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...
  40. 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...
  41. doi 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...
  42. 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...
  43. doi 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)...
  44. 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
    ....
  45. 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) ..
  46. 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...
  47. 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...
  48. 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 ..
  49. 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.
  50. 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...
  51. 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...
  52. 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...
  53. ncbi Creutzfeldt-Jakob disease and inclusion body myositis: abundant disease-associated prion protein in muscle
    Gabor G Kovacs
    Institute of Neurology, University of Vienna, and Austrian Reference Centre for Human Prion Diseases, Vienna, Austria
    Ann Neurol 55:121-5. 2004
    ..we demonstrated abundant PrP(Sc) in the muscle of a patient with sporadic Creutzfeldt-Jakob disease and inclusion body myositis. Extraneural PrP(C)-PrP(Sc) conversion in Creutzfeldt-Jakob disease appears to become prominent when PrP(..
  54. ncbi Coexistence of X-linked recessive Emery-Dreifuss muscular dystrophy with inclusion body myositis-like morphology
    Anna Fidzianska
    Neuromuscular Unit, MRC, Polish Academy of Science, 1a Banacha Str, 02 097 Warsaw, Poland
    Acta Neuropathol 107:197-203. 2004
    ..Two patients demonstrated a typical inclusion body myositis (IBM)-like morphology. The third patient had only minor changes...
  55. 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...
  56. 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...
  57. 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 ..
  58. 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...
  59. 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...
  60. ncbi Inclusion body myositis in connective tissue disorders: case report and review of the literature
    C T Derk
    Thomas Jefferson University Hospital, Pennsylvania, Philadelphia, USA
    Clin Rheumatol 22:324-8. 2003
    ..a patient with systemic lupus erythematosus (SLE) and secondary Sjögren's syndrome (SS) who developed inclusion body myositis (IBM) which, contrary to the typical presentation of this disorder, was symmetrical in nature although ..
  61. pmc Transgenic mice over-expressing the C-99 fragment of betaPP with an alpha-secretase site mutation develop a myopathy similar to human inclusion body myositis
    L W Jin
    Department of Pathology, University of Washington, Seattle 98195 6480, USA
    Am J Pathol 153:1679-86. 1998
    b>Inclusion body myositis (IBM) is the most common muscle disease in the elderly...
  62. 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.
  63. doi 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.
  64. 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...
  65. doi 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...
  66. doi 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.
  67. pmc Absence of characteristic features in two patients with inclusion body myositis
    M F van der Meulen
    Department of Neurology, University Hospital Utrecht, The Netherlands
    J Neurol Neurosurg Psychiatry 64:396-8. 1998
    According to recently published criteria a diagnosis of definite sporadic inclusion body myositis is made if the typical histopathological abnormalities (rimmed vacuoles and abnormal accumulations of proteins, in addition to mononuclear ..
  68. 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...
  69. 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 (..
  70. 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...
  71. 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.
  72. 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)...
  73. 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)...
  74. ncbi High-dose immunoglobulin therapy in sporadic inclusion body myositis: a double-blind, placebo-controlled study
    M C Walter
    Department of Neurology, Friedrich Baur Institut, Medizinische Klink, University of Munich, Germany
    J Neurol 247:22-8. 2000
    Sporadic inclusion body myositis (s-IBM) is an acquired inflammatory muscle disease of unknown cause. In general, s-IBM presents with slowly progressive, asymmetric weakness, and atrophy of skeletal muscle...
  75. ncbi Treatment of inclusion body myositis
    P Cherin
    Service de Médecine Interne du Pr Herson, Paris, France
    Curr Opin Rheumatol 11:456-61. 1999
    Sporadic inclusion body myositis (s-IBM) is considered the most common muscle disease in patients older than 50 years, with a male predominance...
  76. pmc Amyloid-beta deposition in skeletal muscle of transgenic mice: possible model of inclusion body myopathy
    K Fukuchi
    Department of Comparative Medicine, Schools of Medicine and Dentistry, University of Alabama at Birmingham, 35294 0019, USA
    Am J Pathol 153:1687-93. 1998
    ..These mice may be a useful model of inclusion body myopathy, which shares a number of pathological markers with Alzheimer's disease...
  77. ncbi Hereditary inclusion body myopathy: the Middle Eastern genetic cluster
    Z Argov
    Department of Neurology and Agnes Ginges Center for Human Neurogenetics, Hadassah University Hospital and Hebrew University Hadassah Medical School, Jerusalem
    Neurology 60:1519-23. 2003
    ..The recent identification of the gene responsible for this myopathy and the common "Persian Jewish mutation" (M712T) enabled the re-evaluation of atypical phenotypes and the epidemiology of HIBM in various communities in the Middle East...
  78. 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...
  79. doi 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...
  80. 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...
  81. 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...
  82. 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...
  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 Inclusion body myositis evolving in systemic lupus erythrematosus? A case report
    G Massawi
    Rheumatology (Oxford) 42:1012-4. 2003
  86. 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)...
  87. ncbi Polymyositis: an overdiagnosed entity
    Walter G Bradley
    Neurology 63:402; author reply 403. 2004
  88. ncbi [Inflammatory muscle diseases: dermatomyositis, polymyositis, and inclusion body myositis]
    E Genth
    Rheumaklinik und Rheumaforschungsinstitut Aachen
    Internist (Berl) 46:1218-32. 2005
    Dermatomyositis, polymyositis, inclusion body myositis and myositis overlap syndromes are systemic immune disorders of unknown origin with muscle weakness and elevated values of creatinkinase in the serum...
  89. ncbi Dysphagia in inflammatory myopathy: clinical characteristics, treatment strategies, and outcome in 62 patients
    Terry H Oh
    Department of Physical Medicine and Rehabilitation, College of Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA
    Mayo Clin Proc 82:441-7. 2007
    ..To assess the clinical characteristics, treatment, and outcome of patients with inflammatory myopathy-associated dysphagia...
  90. ncbi Major histocompatibility complex class I and II detection as a diagnostic tool in idiopathic inflammatory myopathies
    Ayushi Jain
    Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
    Arch Pathol Lab Med 131:1070-6. 2007
    ..diagnosis of idiopathic inflammatory myopathies (IIMs), which include dermatomyositis, polymyositis, and inclusion body myositis. Currently, there is no definite diagnostic marker that helps in the discrimination of different ..
  91. ncbi Biologics in the treatment of primary inflammatory myositis
    Daniel Wendling
    Joint Bone Spine 74:316-8. 2007
  92. ncbi Myeloid dendritic cells in inclusion-body myositis and polymyositis
    Steven A Greenberg
    Department of Neurology, Division of Neuromuscular Disease, Brigham and Women s Hospital, 75 Francis Street, and Harvard Medical School, Boston, Massachusetts 02115, USA
    Muscle Nerve 35:17-23. 2007
    ..The stellate morphology of myeloid DCs in dense collections of cells that included T cells suggests local intramuscular antigen presentation in IBM and PM...
  93. ncbi Polymyositis: not a unicorn or mythological beast...but maybe a duck?
    John T Kissel
    Neurology 70:414-5. 2008
  94. doi Inflammatory myopathies: evaluation and management
    Steven A Greenberg
    Department of Neurology, Brigham and Women s Hospital, Department of Neurology, Division of Neuromuscular Disease, Brigham and Women sHospital, and Harvard Medical School, Boston, MA 02115, USA
    Semin Neurol 28:241-9. 2008
    The inflammatory myopathies, including dermatomyositis, inclusion body myositis, and polymyositis, are poorly understood autoimmune diseases affecting skeletal muscle...
  95. ncbi Platelet-endothelial cell adhesion molecule-1 and CD146: soluble levels and in situ expression of cellular adhesion molecules implicated in the cohesion of endothelial cells in idiopathic inflammatory myopathies
    Dominique Figarella-Branger
    Laboratoire de Biopathologie de l Adhésion et de la Signalisation, EA 3281, Faculte de Medecine Timone, Universite de la Mediterranee, Marseille, France
    J Rheumatol 33:1623-30. 2006
    ..Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of diseases characterized by chronic inflammation of muscles. We investigated the role of cellular adhesion molecules implicated in the cohesion of endothelial cells in IIM...
  96. ncbi Needle electromyographic findings in 98 patients with myositis
    Paul J Blijham
    Department of Clinical Neurophysiology, Institute of Neurology, Institute of Neurology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    Eur Neurol 55:183-8. 2006
    ..Little is known about the distribution of electromyographic (EMG) abnormalities in myositis even though this is relevant in daily practice...
  97. pmc Expression of granulysin in polymyositis and inclusion-body myositis
    K Ikezoe
    Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3 1 1, Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    J Neurol Neurosurg Psychiatry 77:1187-90. 2006
    ....
  98. ncbi Therapeutic targets in patients with inflammatory myopathies: present approaches and a look to the future
    Marinos C Dalakas
    Neuromuscular Diseases Section, NINDS, NIH, Building 10, Room 4N248, 10 Center Drive MSC 1382, Bethesda, MD 20892 1382, USA
    Neuromuscul Disord 16:223-36. 2006
  99. ncbi Distribution of glucocorticoid receptor alpha and beta subtypes in the idiopathic inflammatory myopathies
    Jan L De Bleecker
    Department of Neurology, Ghent University Hospital, De Pintelaan 185, B 9000 Gent, Belgium
    Neuromuscul Disord 17:186-93. 2007
    In contrast with dermatomyositis and polymyositis, inclusion body myositis is unresponsive to glucocorticoid treatment...
  100. pmc Limited effects of high-dose intravenous immunoglobulin (IVIG) treatment on molecular expression in muscle tissue of patients with inflammatory myopathies
    Sevim Barbasso Helmers
    Rheumatology Unit, Department of Medicine, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden
    Ann Rheum Dis 66:1276-83. 2007
    ..immunoglobulin (IVIG) in inflammatory myopathies by investigating the effects on muscle function and immunological molecules in skeletal muscle of polymyositis (PM), dermatomyositis (DM) and inclusion body myositis (IBM) patients.
  101. ncbi Update on idiopathic inflammatory myopathies
    C Briani
    University of Padova, Department of Neurosciences, Padova, Italy
    Autoimmunity 39:161-70. 2006
    ..features, three major diseases can be identified: dermatomyositis (DM); polymyositis (PM); and inclusion body myositis (IBM)...

Research Grants74

  1. Role of Type I Interferons in a Self-Sustaining Murine Model of Myositis
    Thomas Griffin; Fiscal Year: 2007
    ..myopathies comprise a group of connective tissue diseases that include dermatomyositis, polymyositis, and inclusion body myositis. Each of these conditions is characterized by chronic skeletal muscle inflammation and muscle fiber ..
  2. Role of Presenilin in Idiopathic Dilated Cardiomyopathy
    Federica Del Monte; Fiscal Year: 2010
    ..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. 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...
  4. A Multidisciplinary Analysis of Gelsolin Amyloid Disease
    Jeffery Kelly; Fiscal Year: 2007
    ..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...
  5. Cellular Nucleic Acid Binding Protein (CNBP) in Aging and Disease
    Michael Paul Murphy; Fiscal Year: 2010
    ..Further, both BACE1 and the related peripheral enzyme BACE2 are both increased in Inclusion Body Myositis, an age-related, degenerative disease of the skeletal musculature considered by some to be a pathological ..
  6. Cellular Nucleic Acid Binding Protein (CNBP) in Aging and Disease
    Michael Murphy; Fiscal Year: 2009
    ..Further, both BACE1 and the related peripheral enzyme BACE2 are both increased in Inclusion Body Myositis, an age-related, degenerative disease of the skeletal musculature considered by some to be a pathological ..
  7. Cellular Nucleic Acid Binding Protein (CNBP) in Aging and Disease
    Michael Murphy; Fiscal Year: 2007
    ..Further, both BACE1 and the related peripheral enzyme BACE2 are both increased in Inclusion Body Myositis, an age-related, degenerative disease of the skeletal musculature considered by some to be a pathological ..
  8. Idiopathic Inflammatory Myopathies: Improving Diagnosis and Predicting Outcomes
    Lisa Christopher Stine; Fiscal Year: 2007
    ..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...
  9. 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...
  10. Multiparametric Classification of Muscle Damage in Inflammatory Myopathy
    Jane Park; Fiscal Year: 2010
    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...
  11. Multiparametric Classification of Muscle Damage in Inflammatory Myopathy
    BRUCE DAMON; Fiscal Year: 2009
    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...
  12. Alzheimer Vaccines: Noninvasive Vaccination by DNA-Base*
    Ken Ichiro Fukuchi; Fiscal Year: 2004
    ..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. ..
  13. 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. ..
  14. Pathogenic Role of Abeta, tau and Inflammation in Inclusion Body Myositis
    Masashi Kitazawa; Fiscal Year: 2007
    b>Inclusion body myositis (IBM) is the leading age-related skeletal muscle disorder, yet its etiology remains unknown, nor do effective treatments exist...
  15. 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)...
  16. 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 ..
  17. Role of RyRs and DHPRs and Ca2+ Entry in beta-amyloid mediated Ca2+ dysregulation
    Alexander Shtifman; Fiscal Year: 2007
    ..clinical scientist with extensive experience in adenoviral gene delivery and in the field of the Inclusion Body Myositis (IBM) and Dr...
  18. 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. ..
  19. IBMPFD MUTATIONS IMPAIR UPS FUNCTION
    CONRAD WEIHL; Fiscal Year: 2009
    ..We propose to explore the consequence of disease mutations in p97/VCP on UPS-mediated protein degradation in skeletal muscle and its relevance to aging related disorders such as inclusion body myositis and fronto-temporal dementia.
  20. AAA ATPase p97/VCP and Inclusion Body Myopathy
    CONRAD WEIHL; Fiscal Year: 2007
    ..goal to investigate the cellular mechanisms of aging in relation to skeletal muscle disorders, using inclusion body myositis (IBM) as a prototypical disease...
  21. IBMPFD MUTATIONS IMPAIR UPS FUNCTION
    CONRAD WEIHL; Fiscal Year: 2009
    ..We propose to explore the consequence of disease mutations in p97/VCP on UPS-mediated protein degradation in skeletal muscle and its relevance to aging related disorders such as inclusion body myositis and fronto-temporal dementia.