PRION PROTEIN IS ABNORMALLY ACCUMULATED IN INCLUSION-BODY MYOSITIS

被引：59

作者：

ASKANAS, V ^{[1
]}

BILAK, M ^{[1
]}

ENGEL, WK ^{[1
]}

ALVAREZ, RB ^{[1
]}

TOME, F ^{[1
]}

LECLERC, A ^{[1
]}

机构：

[1] INSERM,U153,PARIS,FRANCE

来源：

NEUROREPORT | 1993年 / 5卷 / 01期

关键词：

PRION PROTEIN; MUSCLE FIBERS; INCLUSION-BODY MYOSITIS;

D O I：

10.1097/00001756-199310000-00006

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

IN muscle biopsies of 8 sporadic inclusion-body myositis (S-IBM) and 4 hereditary inclusion-body myopathy (H-IBM) patients, vacuolated muscle fibers contained within their vacuoles strongly immunoreactive inclusions with 2 polyclonal and 1 monoclonal antibodies against prion protein (PrP). By light-microscopy, PrP deposits co-localized with beta-amyloid protein (A beta) and ubiquitin (Ub). By immuno-electronmicroscopy, both PrP and A beta were present on amorphous material and on 6-10 nm amyloid-like fibrils; and PrP and Ub co-localized on cytoplasmic twisted tubulofilaments (TTFs) and on amorphous material. Our study provides the first demonstration of abnormally accumulated PrP in pathological tissue other than brain, and it suggests that PrP may play a role in the pathogenesis of IBM.

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页码：25 / 28

页数：4

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