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各種ミトコンドリア脳筋症における中枢神経系病変の発生機序

各种线粒体脑肌病中枢神经系统病变的发生机制

基本信息

批准号：
02670357
负责人：
大浜栄作
金额：
$ 1.22万
依托单位：
Niigata University
依托单位国家：
日本
项目类别：
Grant-in-Aid for General Scientific Research (C)
财政年份：
1990
资助国家：
日本
起止时间：
1990 至无数据
项目状态：
已结题

项目摘要

我々はこれまで剖検例の検索によりMELAS(mitochondrial myopathy,encephalopathy,lactic acidosis,and strokeーlike episodes)の脳血管,脈絡叢,脳下垂体前葉に著明なミトコンドリア異常の存在することを見い出し,これらが本症における脳病変の発生と病態に密接に関与していることを明らかにしてきた。本研究では,MELASと共にミトコンドリア脳筋症の代表的疾患であるMERRF(mitochondrial encephalopathy with raggedーred fibers)についても検索を拡げ,下記の新しい知見を得ることができた。1.MERRFにおける脳血管のミトコンドリア異常2剖検例の脳血管系をMELASにおけると同様に検索した結果,血統平滑筋組胞,内皮細胞に軽度ながらMELASと同質のミトコンドリア異常の存在することを見い出した。この所見はMERRFの症例の約30%に認められるとされている脳卆中様症状の発生に関与していると考えられる。2.MERRFにおける脳下垂体前葉のミトコンドリア異常MERRFの脳下垂体前葉にも,MELASと全く同質の変化が,同程度に存在することが判明した。この変化は本症患者に共通する低身長や発育不全,および種々の内分泌異常の原因をなすと考えられる。3.MELASおよびMERRFにおけるミトコンドリア心筋症各々2例の剖検心の検索の結果,心筋細胞の著明なミトコンドリア異常,筋原線維の減少,心筋組胞の変性が存在し,このため心臓は求心性肥大を来たすことを明らかにし得た。4.MELASおよびMERRFの外眼筋のミトコンドリア異常MELASおよびMERRFでは,骨格筋や心筋,および血管平滑筋とならんで外眼筋にも著明なミトコンドリア異常の存在を明らかにした。

In our hospital, the MELAS (mitochondrial myopathy,encephalopathy,lactic acidosis,and stroke like episodes) blood vessels were dissected, and the anterior pituitary gland was clearly marked by the presence of convulsions of the blood vessels and the anterior pituitary gland. In this study, MELAS patients were divided into two groups: MERRF (mitochondrial encephalopathy with ragged syndrome red fibers), which is the representative of the disease of tendon disease. The results showed that the blood system smooth muscle tissue was smooth, the endothelial cell density (MELAS) was similar to that of the endothelial cell (MELAS), and there were significant differences between the vascular system and the vascular system. The results showed that the blood system smooth muscle cells, endothelial cells and endothelial cells were the same. There are about 30% of the cases of MERRF in the institute. The anterior pituitary gland was often detected by MERRF, MELAS was homogenized, and the same degree was detected in the same degree. It is common for patients with this disease to have low body height and poor fertility, and there are many common causes of endocrine disorders. There were 2 cases of cardiomyopathy and 2 cases of cardiomyopathy in each group of 3.MELAS. The results showed that the heart muscle cells showed that the heart muscle was abnormal, the muscle tissue was low, and the heart tissue was weak. In order to improve the cardiac hypertrophy, the results showed that the heart muscle cells were normal, the original tendons were low, and the cardiac tissue was not normal. 4.MELAS, MERRF, external eye tendons, MERRF tendons, bone tendons, blood vessels, smooth tendons, external eye tendons, MERRF tendons, blood vessels, smooth tendons, blood vessels, smooth tendons, smooth tendons, smooth