Human Xenomitochondrial Cybrids
Human Xenomitochondrial Cybrids
复制标题
人类异种线粒体杂种
DOI：
--
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发表时间：
1998
影响因子：
4.8
通讯作者：
C. Moraes
中科院分区：
文献类型：
--
作者：
A. Barrientos;L. Kenyon;C. Moraes
The subunits forming the mitochondrial oxidative phosphorylation system are coded by both nuclear and mitochondrial genes. Recently, we attempted to introduce mtDNA from non-human apes into a human cell line lacking mtDNA (ρ°), and succeeded in producing human-common chimpanzee, human-pigmy chimpanzee, and human-gorilla xenomitochondrial cybrids (HXC). Here, we present a comprehensive characterization of oxidative phosphorylation function in these cells. Mitochondrial complexes II, III, IV, and V had activities indistinguishable from parental human or non-human primate cells. In contrast, a complex I deficiency was observed in all HXC. Kinetic studies of complex I using decylubiquinone or NADH as limiting substrates showed that the V max was decreased in HXC by approximately 40%, and the K m for the NADH was significantly increased (3-fold, p < 0.001). Rotenone inhibition studies of intact cell respiration and pyruvate-malate oxidation in permeabilized cells showed that 3 nm rotenone produced a mild effect in control cells (0–10% inhibition) but produced a marked inhibition of HXC respiration (50–75%). Immunoblotting analyses of three subunits of complex I (ND1, 75 and 49 kDa) showed that their relative amounts were not significantly altered in HXC cells. These results establish HXC as cellular models of complex I deficiency in humans and underscore the importance of nuclear and mitochondrial genomes co-evolution in optimizing oxidative phosphorylation function.
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DOI：
10.1073/pnas.94.26.14900
发表时间：
1997-12-23
影响因子：
11.1
作者：
Wallace, DC;Stugard, C;Brown, MD
通讯作者：
Brown, MD
线粒体肌病和乳酸性酸中毒患者缺乏泛醌细胞色素 C 还原酶。
DOI：
10.1073/pnas.80.16.5103
发表时间：
1983
影响因子：
11.1
作者：
Darley-Usmar,VM;Kennaway,NG;Buist,NR;Capaldi,RA
通讯作者：
Capaldi,RA
影响因子：
56.9
作者：
WALLACE, DC;SINGH, G;NIKOSKELAINEN, EK
通讯作者：
NIKOSKELAINEN, EK
DOI：
10.1073/pnas.94.26.14894
发表时间：
1997-12-23
影响因子：
11.1
作者：
Hirano, M;Shtilbans, A;Schon, EA
通讯作者：
Schon, EA