权益分类	功能权益	普通用户	{{item.name}}会员
{{category.name}}	{{benefitItem.name}}

IMMUNOCHEMICAL AND PROTEINOLOGICAL STUDY IN THE HEREDITARY MYOPATHY OF THE DIAPHRAGMATIC MUSCLES IN CATTLE

牛遗传性膈肌肌病的免疫化学和蛋白质学研究

基本信息

批准号：
11660307
负责人：
FURUOKA Hidefumi
金额：
$ 2.3万
依托单位：
OBIHIRO UNIVERSITY OF AGRICULTURE & VATERINARY MEDICINE
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (C)
财政年份：
1999
资助国家：
日本
起止时间：
1999 至 2000
项目状态：
已结题

来源：
https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-11660307/
关键词：
CATTLE HEREDITY MUSCULAR DISEASE MUSUCULAR DYSTROPHY PATHOLOGY DIAPHRAGMATIC MUSCLE CARDIOMYOPATHY

项目摘要

In several cases of the cattle which were submitted to the necropsy due to the various diseases, numerous core-like structures without accompanying muscle degeneration have been found in the diaphragmatic and intercostal muscles, and myocardium. Of the 213 heads studied from 8 months to 15 years old, 49 cases were affected this disorder. Addition to these pathological findings which showed a typical the core-like structures seen in the hereditary myopathy of the diaphragmatic muscles, the 49 cases were deribed from the same family line in the cases of this myopathy. These observations suggested that the 49 cases might have the potency of this myopathy.In order to investigate the pathogenesis of this myopathy, antibodies for the both diseased and normal muscle tissue, respectively, were prepared for the immunoblotting analysis. The immunoblots of homogenates of the diseased muscles demonstrated the presence of two specific bands (approximately 55 and 65 kDa) and decreased level of immunoreactivity (about 85 kDa). Although this study failed to reach the identification of these abnormal proteins, they seemed to play some part in the pathogenesis of this myopathy.Since the late 1990s, we encountered a considerable number of cases of the dilated cardiomyopathy. By histology, the myocardium revealed hypertrophy and vacuolation of the cardiac muscle fibers and severe fibrosis. Pedigree analysis of affected cattle indicated an autosomal recessive mode of inheritance. The family line of this cardiomyopathy overlapped with the one of the hereditary myopathy of the diaphragmatic muscles in Holstein-Friesian cattle, although those disorders exhibit a varying pathology. The genetic basis may support those different phenotypes appearing from one family tree.

在因各种疾病而进行尸检的几例牛中，在胸肌、肋间肌和心肌中发现了许多核心样结构，但未伴随肌肉变性。在213例8个月至15岁的儿童中，有49例患有这种疾病。除此之外，病理学检查显示出典型的核心样结构，这49例患者均来自同一家系。为探讨该肌病的发病机制，我们分别制备了病肌和正常肌组织的抗体，进行免疫印迹分析。患病肌肉匀浆的免疫印迹显示存在两个特异性条带（约55和65 kDa）和降低的免疫反应性水平（约85 kDa）。虽然本研究未能鉴定出这些异常蛋白，但它们似乎在扩张型心肌病的发病机制中发挥了一定作用。自20世纪90年代末以来，我们遇到了相当数量的扩张型心肌病病例。通过组织学检查，心肌显示心肌纤维肥大和空泡化以及严重纤维化。受影响的牛的系谱分析表明，常染色体隐性遗传方式。这种心肌病的家系与Holstein-Friesian牛的遗传性肌肉萎缩性肌病重叠，尽管这些疾病表现出不同的病理学。遗传基础可能支持从一个家谱中出现的那些不同的表型。