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培养的包涵体肌炎肌纤维不会积累β-淀粉样前体蛋白,并且可以被神经支配。

Cultured inclusion-body myositis muscle fibers do not accumulate beta-amyloid precursor protein and can be innervated.

作者信息

McFerrin J, Engel W K, Askanas V

机构信息

USC Neuromuscular Center, Department of Neurology, University of Southern California Keck School of Medicine, Good Samaritan Hospital, Los Angeles 90017-1912, USA.

出版信息

Neurology. 1999 Dec 10;53(9):2184-7. doi: 10.1212/wnl.53.9.2184.

DOI:10.1212/wnl.53.9.2184
PMID:10599804
Abstract

Cultured muscle fibers from patients with sporadic inclusion-body myositis (s-IBM), similar to normal control muscle fibers, 1) did not have beta-amyloid precursor protein (betaAPP) immunoreactivity; and 2) became normally innervated, as evidenced by the following features: full cross-striation, continuous contraction, and acetylcholinesterase and acetylcholine receptors accumulated only at neuromuscular junctions. Thus, factors responsible for betaAPP accumulation and denervation-like changes in s-IBM muscle biopsies are not operative in the relatively short-term, non-aged, cultured s-IBM muscle fibers.

摘要

散发性包涵体肌炎(s-IBM)患者的培养肌纤维,与正常对照肌纤维相似,1)没有β-淀粉样前体蛋白(βAPP)免疫反应性;2)神经支配正常,表现为以下特征:横纹完整、持续收缩,且乙酰胆碱酯酶和乙酰胆碱受体仅在神经肌肉接头处聚集。因此,导致s-IBM肌肉活检中βAPP积累和去神经样改变的因素,在相对短期、未老化的培养s-IBM肌纤维中不起作用。

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