Inclusion-body myositis: newest concepts of pathogenesis and relation to aging and Alzheimer disease.

Abstract:

:We review the newest advances related to seeking the pathogenic mechanism(s) of sporadic inclusion-body myositis (s-IBM) and present the pathologic diagnostic criteria of s-IBM. We discuss the possible pathogenic role of several themes, such as 1) increased amyloid-beta precursor protein (AbetaPP) and of its fragment Abeta; 2) phosphorylation of tau protein; 3) oxidative stress; 4) abnormal a) signal-transduction, b) transcription, and c) RNA accumulation; 5) "junctionalization" and myogenous" denervation; and 6) lymphocytic inflammation. Evidence is provided supporting our hypothesis that overexpression of AbetaPP within the aging muscle fibers is an early upstream event causing the subsequent pathogenic cascade. The remarkable pathologic similarities between s-IBM muscle and Alzheimer disease (AD) brain are discussed, and the possible cause and significance are addressed.

authors

Askanas V,Engel WK

doi

10.1093/jnen/60.1.1

subject

Has Abstract

pub_date

2001-01-01 00:00:00

pages

1-14

issue

1

eissn

0022-3069

issn

1554-6578

journal_volume

60

pub_type

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