复合物 I 生物发生
中文名称
通路描述
复合物 I(烟酰胺腺嘌呤二核苷酸:泛醌氧化还原酶或烟酰胺腺嘌呤二核苷酸脱氢酶)利用糖酵解和三羧酸循环产生的 NADH 将质子泵出线粒体基质。它是电子传递链中最大的酶复合物,包含 11 个核心亚基和 34 个辅助亚基。七个亚基(ND1-6、ND4L)由线粒体 DNA 编码,其余由核 DNA 编码。该酶含有 FMN 辅基和 8 个铁硫(Fe-S)簇。亚基通过预组装的亚复合物以协调的方式组装形成成熟的全酶。至少 24 种所谓的“组装因子”蛋白,以内在或暂时性方式起作用,对于构建复合物 I 是必需的,尽管它们在生物发生中的确切作用尚未完全理解(Fernandez-Vizarra 等人,2009;Mckenzie 和 Ryan 2010;Mimaki 等人,2012;Andrews 等人,2013;综述 Laube 等人,2024)。
英文描述
Complex I biogenesis Complex I (NADH:ubiquinone oxidoreductase or NADH dehydrogenase) utilises NADH formed from glycolysis and the TCA cycle to pump protons out of the mitochondrial matrix. It is the largest enzyme complex in the electron transport chain, containing 11 core and 34 accessory subunits. Seven subunits (ND1-6, ND4L) are encoded by mitochondrial DNA, the remainder are encoded in the nucleus. The enzyme has a FMN prosthetic group and 8 Iron-Sulfur (Fe-S) clusters. The subunits are assembled together in a coordinated manner via preassembled subcomplexes to form the mature holoenzyme. At least 24 so-called "assembly factor" proteins, acting intrinsically or transiently, are required for constructing complex I although their exact roles in the biogenesis are not fully understood (Fernandez-Vizarra et al. 2009, Mckenzie & Ryan 2010, Mimaki et al. 2012, Andrews et al. 2013; reviewed by Laube et al., 2024).
所含基因
63 个基因
ACAD9
COA1
DMAC1
DMAC2
ECSIT
FOXRED1
HSCB
HSPA9
LYRM2
MT
NDUFA1
NDUFA10
NDUFA11
NDUFA12
NDUFA13
NDUFA2
NDUFA3
NDUFA5
NDUFA6
NDUFA7
NDUFA8
NDUFA9
NDUFAB1
NDUFAF1
NDUFAF2
NDUFAF3
NDUFAF4
NDUFAF5
NDUFAF6
NDUFAF7
NDUFAF8
NDUFB1
NDUFB10
NDUFB11
NDUFB2
NDUFB3
NDUFB4
NDUFB5
NDUFB6
NDUFB7
NDUFB8
NDUFB9
NDUFC1
NDUFC2
NDUFS1
NDUFS2
NDUFS3
NDUFS4
NDUFS5
NDUFS6
NDUFS7
NDUFS8
NDUFV1
NDUFV2
NDUFV3
NUBPL
OXA1L
PYURF
SFXN4
TIMMDC1
TMEM126A
TMEM126B
TMEM186