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发现水通道在癫痫和杜氏肌营养不良症中的影响的优先事项。

Priorities in the discovery of the implications of water channels in epilepsy and duchenne muscular dystrophy.

作者信息

Benga I

机构信息

Department of Pediatric Neurology, Iuliu Hatieganu University of Medicine and Pharmacy Cluj-Napoca, Romania.

出版信息

Cell Mol Biol (Noisy-le-grand). 2006 Oct 30;52(7):46-50.

Abstract

In addition to the priority in the discovery of the first water channel protein in the red blood cell membrane the group of Gheorghe Benga in Cluj-Napoca, Romania, also has a world priority in the discovery of the implications of water channel proteins in epilepsy and Duchenne muscular dystrophy. This priority is briefly presented here. In 1977 Benga and Morariu reported a decreased water permeability of red blood cells in children with idiopathic epilepsy (cases selected by Ileana Benga). This investigation was performed as part of a program of research of hydroelectrolytic alterations in child epilepsy. On the other hand the group of Gheorghe Benga has reported a decreased water permeability of RBC in patients with Duchenne muscular dystrophy. These findings were interpreted as an expression of generalized membrane defects affecting water permeability in epilepsy and Duchenne muscular dystrophy. In recent years this idea was confirmed by reports indicating aquaporin abnormalities in the brain of epileptic patients and in the muscle of Duchenne muscular dystrophy patients.

摘要

除了在发现红细胞膜上首个水通道蛋白方面具有优先权外,罗马尼亚克卢日 - 纳波卡的格奥尔基·本加团队在发现水通道蛋白与癫痫和杜兴氏肌肉营养不良症的关联方面也在世界上具有优先权。在此简要介绍这一优先权。1977年,本加和莫拉里乌报告称,特发性癫痫患儿(由伊利安娜·本加挑选病例)的红细胞水通透性降低。这项研究是儿童癫痫水电解质改变研究项目的一部分。另一方面,格奥尔基·本加团队报告了杜兴氏肌肉营养不良症患者红细胞的水通透性降低。这些发现被解释为癫痫和杜兴氏肌肉营养不良症中影响水通透性的全身性膜缺陷的一种表现。近年来,有报告表明癫痫患者大脑和杜兴氏肌肉营养不良症患者肌肉中存在水通道蛋白异常,这证实了这一观点。

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