Molecular pathology and pathogenesis of inclusion-body myositis

被引：27

作者：

Askanas, V ^{[1
]}

Engel, WK ^{[1
]}

机构：

[1] USC, Neuromuscular Ctr, Good Samaritan Hosp, Dept Neurol,Keck Sch Med, Los Angeles, CA 90017 USA

来源：

MICROSCOPY RESEARCH AND TECHNIQUE | 2005年 / 67卷 / 3-4期

关键词：

amyloid-beta; amyloid-beta precursor protein; phosphorylated tau; misfolded proteins; protein aggregates;

D O I：

10.1002/jemt.20186

中图分类号：

R602 [外科病理学、解剖学]; R32 [人体形态学];

学科分类号：

100101 ;

摘要：

We summarize the molecular phenotype, diagnostic criteria, and the newest advances related to seeking the pathogenic mechanism(s) of sporadic inclusion-body myositis (s-IBM), a muscle disease usually of persons over age 50. On the basis of our research, several processes seem to be important in relation to the still-speculative pathogenesis: 1) increased transcription and accumulation of amyloid-P precursor protein (APPP), and accumulation of its proteolytic fragment A beta; 2) abnormal accumulation of cholesterol, caveolin-1, and apolipoprotein E; 3) oxidative stress; 4) accumulations of intramusele fiber multiprotein aggregates; and 5) evidence that unfolded/misfolded proteins participate in s-IBM pathogenesis. Our basic hypothesis is that overexpression of APPP within the aging muscle fibers is an early upstream event causing a subsequent pathogenic cascade.

引用

页码：114 / 120

页数：7

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