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• W2019957886 abstract "Sporadic inclusion-body myositis (s-IBM), the most common muscle disease of older persons, is of unknown cause and there is no successful treatment. We summarize our most recent findings, which provide a better understanding of the steps in the pathogenetic cascade. We suggest that s-IBM is primarily a myodegenerative disease. Intriguing are the phenotypic similarities between s-IBM muscle fibers and the brains of Alzheimer disease, the most common neurodegenerative disease of older persons. In s-IBM, abnormal accumulation of the amyloid-beta (Abeta) precursor protein and its proteolytic fragment, Abeta, associated with the aging intracellular milieu of the muscle fiber, appear to be key upstream pathogenic events. We propose that the identified abnormal accumulation, misfolding, and aggregation of proteins, perhaps provoked by the aging milieu and aggravated by the oxidative stress, lead to the s-IBM-specific vacuolar degeneration and atrophy of muscle fibers." @default.
• W2019957886 created "2016-06-24" @default.
• W2019957886 creator A5008326837 @default.
• W2019957886 creator A5029701142 @default.
• W2019957886 date "2005-12-16" @default.
• W2019957886 modified "2023-10-14" @default.
• W2019957886 title "Inclusion-body myositis: A myodegenerative conformational disorder associated with A , protein misfolding, and proteasome inhibition" @default.
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• W2019957886 doi "https://doi.org/10.1212/01.wnl.0000192128.13875.1e" @default.
• W2019957886 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/16432144" @default.
• W2019957886 hasPublicationYear "2005" @default.
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