The ABC transporter Atm1p is required for mitochondrial iron homeostasis
The ABC transporter Atm1p is required for mitochondrial iron homeostasis
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DOI：
10.1016/s0014-5793(97)01414-2
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发表时间：
1997-12-01
期刊：
影响因子：
3.5
通讯作者：
Lill, R
中科院分区：
文献类型：
--
作者：
Kispal, G;Csere, P;Lill, R
位于线粒体内膜的IBC转运蛋白Atm1p的功能尚不清楚。为了研究其细胞作用，我们分析了ATM1被破坏的突变体，Delta atm1细胞缺乏线粒体内外的血红素携带蛋白的全形物，但不缺乏血红素携带蛋白的脱辅基物，然而血红素的合成和运输都是功能性的，Delta atm1细胞对氧化试剂存在下的生长过敏，并且它们含有增加水平的抗氧化剂谷胱甘肽，特别是其氧化形式，Atm1p缺陷的线粒体与野生型细胞器相比积累30倍高水平的游离铁，即比Frataxin缺陷的线粒体多3倍，Frataxin是Friedreich共济失调中突变的蛋白质。线粒体铁含量增加可能是Δ Atm1细胞中含血红素蛋白的氧化损伤的原因，我们的数据分配一个重要的功能Atm1p在线粒体铁稳态。(C)1997年欧洲生物化学学会联合会。
The function of the,IBC transporter Atm1p located in the mitochondrial inner membrane is not Set known. To study its cellular role, we analyzed a mutant in which ATM1 was disrupted, Delta atm1 cells are deficient in the holoforms, but not the apoforms of heme-carrying proteins both within and outside mitochondria, yet both synthesis and transport of heme are functional, Delta atm1 cells are hypersensitive for growth in the presence of oxidative reagents, and they contain increased levels of the antioxidant glutathione, in particular of its oxidized form, Mitochondria deficient in Atm1p accumulate 30-fold higher levels of free iron as compared to wild-type organelles, i.e. threefold more than mitochondria deficient in frataxin, the protein mutated in Friedreich's ataxia, The increased mitochondrial iron content may be causative of the oxidative damage of heme-containing proteins in Delta atm1 cells, Our data assign an important function to Atm1p in mitochondrial iron homeostasis. (C) 1997 Federation of European Biochemical Societies.