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线粒体菱形蛋白酶:从默默无闻走向疾病相关基因巅峰的历程。

The mitochondrial rhomboid protease: its rise from obscurity to the pinnacle of disease-relevant genes.

作者信息

Chan Eliana Y L, McQuibban G Angus

机构信息

Department of Biochemistry, University of Toronto, Toronto, Ontario, Canada.

出版信息

Biochim Biophys Acta. 2013 Dec;1828(12):2916-25. doi: 10.1016/j.bbamem.2013.05.012.

DOI:10.1016/j.bbamem.2013.05.012
PMID:24099009
Abstract

The Rhomboid proteases belong to a highly conserved family of proteins that are present in all branches of life. In Drosophila, the secretory pathway-localized rhomboid proteases are crucial for epidermal growth factor (EGF) signaling. The identification of a mitochondrial-localized rhomboid protease shed light on other functions of rhomboid proteases including the maintenance of mitochondrial morphology and the regulation of apoptosis. More recent work has revealed other functions of the mitochondrial rhomboid protease in mitochondrial and cellular biology, failure of which have been implicated in human diseases. In this review, we will summarize the current knowledge and disease relevance of the mitochondrial-localized rhomboid protease. This article is part of a Special Issue entitled: Intramembrane Proteases.

摘要

类菱形蛋白酶属于一类高度保守的蛋白质家族,存在于生命的各个分支中。在果蝇中,定位于分泌途径的类菱形蛋白酶对表皮生长因子(EGF)信号传导至关重要。一种定位于线粒体的类菱形蛋白酶的鉴定揭示了类菱形蛋白酶的其他功能,包括维持线粒体形态和调节细胞凋亡。最近的研究揭示了线粒体类菱形蛋白酶在线粒体和细胞生物学中的其他功能,其功能障碍与人类疾病有关。在这篇综述中,我们将总结定位于线粒体的类菱形蛋白酶的现有知识及其与疾病的相关性。本文是名为“膜内蛋白酶”的特刊的一部分。

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