Mitochondrial ABC transporters
Mitochondrial ABC transporters
复制标题
DOI：
10.1016/s0923-2508(01)01204-9
复制
发表时间：
2001-04-01
影响因子：
2.6
通讯作者：
Kispal, G
中科院分区：
文献类型：
--
作者：
Lill, R;Kispal, G
与细菌不同的是，线粒体的内膜只有少量的三磷酸腺苷结合盒(ABC)转运蛋白。已知的线粒体ABC蛋白分为两大类，在酿酒酵母中，以半转运蛋白Atm1p和两个同源蛋白Mdl1p和Mdl2p为代表。在人类中，两个Atm1p同源物(ABC7和MTABC3)和两个与MDL1/2p同源的蛋白质已定位于线粒体。类Atm1p蛋白在线粒体铁稳态和胞质中Fe/S蛋白的成熟过程中发挥重要作用。ABC7基因突变是遗传性X连锁铁粒母细胞性贫血和小脑性共济失调(XLSA/A)的原因。MTABC3可能是致死性新生儿综合征的候选基因。线粒体Mdl1/2p样蛋白的功能目前尚不清楚，但值得注意的是，小鼠ABC-Me可能将血红素生物合成的中间产物从基质运输到红系组织的胞浆中。(C)2001年版《爱思唯尔科学与医学》。
In contrast to bacteria, mitochondria contain only a few ATP binding cassette (ABC) transporters in their inner membrane. The known mitochondrial ABC proteins fall into two major classes that, in the yeast Saccharomyces cerevisiae, are represented by the half-transporter Atm1p and the two closely homologous proteins Mdl1p and Mdl2p. In humans two Atm1p orthologues (ABC7 and MTABC3) and two proteins homologous to Mdl1/2p have been localized to mitochondria. The Atm1p-like proteins perform an important function in mitochondrial iron homeostasis and in the maturation of Fe/S proteins in the cytosol. Mutations in ABC7 are causative of hereditary X-linked sideroblastic anemia and cerebellar ataxia (XLSA/A). MTABC3 may be a candidate gene for the lethal neonatal syndrome. The function of the mitochondrial Mdl1/2p-like proteins is not clear at present with the notable exception of murine ABC-me that may transport intermediates of heme biosynthesis from the matrix to the cytosol in erythroid tissues. (C) 2001 Editions scientifiques et medicales Elsevier SAS.