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Cristae formation

Reactome ID: R-HSA-8949613

中文名称

嵴形成

通路描述

嵴是内质膜的内陷,延伸至基质中,并覆盖有细胞色素复合物和 F1Fo ATP 合酶复合物。嵴增加了内质膜的表面积,允许更多的呼吸复合物。嵴也被认为是“质子口袋”,以生成局部膜电势更高的区域。线粒体嵴的生物发生步骤尚未完全阐明(综述 Zick 等 2009),但线粒体接触位点和嵴组织系统的形成(MICOS,以前也称为 MINOS,综述 Rampelt 等 2016, Kozjak-Pavlovic 2016, van der Laan 等 2016)以及线粒体内膜基部的局部卡介磷脂浓度已知的定义内质膜基部的向内弯曲。MICOS 还将这些区域与嵌入外膜的复合物(SAM 复合物和真菌中的 TOM 复合物)联系起来。MICOS 的 CHCHD3(MIC19)和 IMMT(MIC60)亚基也与内膜中的 OPA1 相互作用(Darshi 等 2011, Glytsou 等 2016)。F1Fo ATP 合酶复合物二聚体或寡聚体的形成导致嵴顶部的内质膜极端弯曲(综述 Seelert 和 Dencher 2011, Habersetzer 等 2013)。MICOS 或 F1Fo ATP 合酶寡聚体缺陷产生异常的线粒体形态。
英文描述
Cristae formation Cristae are invaginations of the inner mitochondrial membrane that extend into the matrix and are lined with cytochrome complexes and F1Fo ATP synthase complexes. Cristae increase the surface area of the inner membranes allowing greater numbers of respiratory complexes. Cristae are also believed to serve as "proton pockets" to generate localized regions of higher membrane potential. The steps in the biogenesis of cristae are not yet completely elucidated (reviewed in Zick et al. 2009) but the formation of the Mitochondrial Contact Site and Cristae Organizing System (MICOS, formerly also known as MINOS, reviewed in Rampelt et al. 2016, Kozjak-Pavlovic 2016, van der Laan et al. 2016) and localized concentrations of cardiolipin are known to define the inward curvature of the inner membrane at the bases of cristae. MICOS also links these regions of the inner membrane with complexes (the SAM complex and, in fungi, the TOM complex) embedded in the outer membrane. CHCHD3 (MIC19) and IMMT (MIC60) subunits of MICOS also interact with OPA1 at the inner membrane (Darshi et al. 2011, Glytsou et al. 2016).
Formation of dimers or oligomers of the F1Fo ATP synthase complex causes extreme curvature of the inner membrane at the apices of cristae (reviewed in Seelert and Dencher 2011, Habersetzer et al. 2013). Defects in either MICOS or F1Fo ATP synthase oligomerization produce abnormal mitochondrial morphologies.

所含基因

25 个基因