STRONG IMMUNOREACTIVITY OF ALPHA-1-ANTICHYMOTRYPSIN COLOCALIZES WITH BETA-AMYLOID PROTEIN AND UBIQUITIN IN VACUOLATED MUSCLE-FIBERS OF INCLUSION-BODY MYOSITIS

被引:46
作者
BILAK, M [1 ]
ASKANAS, V [1 ]
ENGEL, WK [1 ]
机构
[1] UNIV SO CALIF,USC NEUROMUSCULAR CTR,SCH MED,DEPT NEUROL,637 S LUCAS AVE,LOS ANGELES,CA 90017
关键词
ALPHA-1-ANTICHYMOTRYPSIN; BETA-AMYLOID PROTEIN; UBIQUITIN; INCLUSION-BODY MYOSITIS;
D O I
10.1007/BF00334447
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
In 10 of 10 inclusion-body myositis (IBM) patients, including 1 hereditary case, vacuolated muscle fibers contained large or small cytoplasmic inclusions immunoreactive for alpha1-antichymotrypsin (alpha1-ACT). All IBM muscle biopsies had characteristic cytoplasmic tubulo-filaments by electron microscopy. None of 17 control muscle biopsies contained the alpha1-ACT immunoreactive inclusions characteristic of IBM. In vacuolated muscle fibers, alpha1-ACT immunoreactive inclusions colocalized with beta-amyloid protein and ubiquitin immunoreactivities. Our study provides the first demonstration of alpha1-ACT accumulations in abnormal human muscle, and it suggest that, as in Alzheimer's disease and Down's syndrome, alpha1-ACT may be involved in the pathogenesis of IBM.
引用
收藏
页码:378 / 382
页数:5
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