Ion pores made of mitochondrial ATP synthase subunit c in the neuronal plasma membrane and Batten disease

被引：15

作者：

McGeoch, JEM

Palmer, DN

机构：

[1] Harvard Univ, Dept Cellular & Mol Biol, Cambridge, MA 02138 USA

[2] Lincoln Univ, Anim & Food Sci Div, Canterbury, New Zealand

来源：

MOLECULAR GENETICS AND METABOLISM | 1999年 / 66卷 / 04期

关键词：

subunit c; ATP synthase; ion pore; neuronal ceroid lipofuscinosis; Batten disease;

D O I：

10.1006/mgme.1999.2822

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

A hypothesis is outlined that the neurodegeneration of the Batten disease syndromes that involve an overaccumulation of subunit c is caused by a newly characterized function of the protein, its ability to assemble in the plasma membrane into ion pores (J. E. M. McGeoch and G. Guidotti, Brain Res 766: 188-194, 1997), rendering the cell liable to constant electrical excitability to a degree that causes cell death. (C) 1999 Academic Press.

引用

页码：387 / 392

页数：6

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