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伴有肌营养不良蛋白缺乏的猫肌肉萎缩症

Feline muscular dystrophy with dystrophin deficiency.

作者信息

Carpenter J L, Hoffman E P, Romanul F C, Kunkel L M, Rosales R K, Ma N S, Dasbach J J, Rae J F, Moore F M, McAfee M B

机构信息

Department of Pathology, MSPCA/Angell Memorial Animal Hospital, Boston 02130.

出版信息

Am J Pathol. 1989 Nov;135(5):909-19.

Abstract

This is the first description of a dystrophin-deficient muscular dystrophy in domestic cats. The disorder appears to be of X-linked inheritance because it affected both males of a litter of four kittens. Immunoblotting and immunofluorescent detection of dystrophin showed dystrophin present in control cat muscle but no detectable dystrophin in either affected cat. The feline muscular dystrophy was progressive and histopathologically resembled human Duchenne/Becker muscular dystrophy except for the lack of fat infiltration and the presence of prominent hypertrophy of both muscle fibers and muscles groups in the feline disorder.

摘要

这是对家猫中一种肌营养不良蛋白缺乏型肌肉萎缩症的首次描述。这种疾病似乎是X连锁遗传,因为一窝四只小猫中的两只雄性都受到了影响。对肌营养不良蛋白进行免疫印迹和免疫荧光检测显示,对照猫的肌肉中存在肌营养不良蛋白,但两只患病猫中均未检测到肌营养不良蛋白。猫的肌肉萎缩症呈进行性发展,组织病理学上类似于人类的杜兴氏/贝克氏肌肉萎缩症,只是猫的疾病中缺乏脂肪浸润,且肌肉纤维和肌肉群有明显的肥大现象。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/22b3/1880103/b8f079774482/amjpathol00119-0140-a.jpg

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