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首页> 外文期刊>Journal of Molecular Biology >Identification and characterization of a mammalian mitochondrial ATP-binding cassette membrane protein.
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Identification and characterization of a mammalian mitochondrial ATP-binding cassette membrane protein.

机译：哺乳动物线粒体ATP结合盒式膜蛋白的鉴定和表征。

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摘要

Membrane proteins of the ATP-binding cassette (ABC) superfamily are involved in the transport of diverse substrates across organellar and plasma membranes of the mammalian cell. Most human ABC proteins identified to date are associated with genetically linked diseases or clinically relevant phenotypes. We describe a new human half-molecule ABC protein, designated M-ABC1, that contains a predicted single membrane and ATP-binding cassette domain. M-ABC1 is localized to membranes of the mitochondria and its transcript is expressed in all tissues. The N-terminal region of the M-ABC1 protein was shown to function independently as a mitochondrial signal sequence by its ability to target the green fluorescent protein to the mitochondria. The monomeric 60 kDa M-ABC1 protein was chemically crosslinked in vivo into a major protein species of 120-130 kDa, thereby confirming that M-ABC1 exists within a higher ordered ABC protein complex. A dominant negative repression approach using M-ABC1 protein with site-directed mutations in its Walker A motif revealed that the mutant protein was rapidly degraded and indicated that the intact Walker A motif of M-ABC1 was required for its stability. The identification of M-ABC1 extends the known distribution of members of the ABC protein family into the mammalian mitochondrion. Copyright 1999 Academic Press.
机译：ATP结合盒（ABC）超家族的膜蛋白参与跨哺乳动物细胞的细胞器和质膜的多种底物运输。迄今为止，大多数人类ABC蛋白都与遗传性疾病或临床相关表型有关。我们描述了一种新的人类半分子ABC蛋白，称为M-ABC1，其中包含一个预测的单膜和ATP结合盒结构域。 M-ABC1位于线粒体膜上，其转录本在所有组织中都有表达。 M-ABC1蛋白的N末端区域通过将绿色荧光蛋白靶向线粒体的能力显示其独立地充当线粒体信号序列。单体60 kDa M-ABC1蛋白在体内化学交联成120-130 kDa的主要蛋白质，从而证实M-ABC1存在于较高序的ABC蛋白复合物中。使用在其Walker A基序中具有定点突变的M-ABC1蛋白的显性负抑制方法表明，突变蛋白迅速降解，表明完整的M-ABC1 Walker A基序是其稳定性所必需的。 M-ABC1的鉴定将ABC蛋白家族成员的已知分布扩展到了哺乳动物的线粒体中。版权所有1999，学术出版社。

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