Pathway
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).
REACT:R-HSA-6799198Reactome
What the reference databases state
Statements
67 statements
67 statements · 1 reference database · Subject: Complex I biogenesis · Sources: Reactome
- ACAD9 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- COA1 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- DMAC1 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- DMAC2 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- ECSIT participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- FOXRED1 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- HSCB participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- HSPA9 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- LYRM2 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- MT-ND1 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- MT-ND2 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- MT-ND3 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
Show the remaining 55
- MT-ND4 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- MT-ND5 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- MT-ND6 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFA1 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFA10 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFA11 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFA12 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFA13 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFA2 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFA3 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFA5 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFA6 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFA7 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFA8 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFA9 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFAB1 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFAF1 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFAF2 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFAF3 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFAF4 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFAF5 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFAF6 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFAF7 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFB1 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFB10 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFB11 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFB2 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFB3 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFB4 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFB5 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFB6 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFB7 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFB8 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFB9 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFC1 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFC2 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFS1 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFS2 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFS3 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFS4 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFS5 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFS6 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFS7 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFS8 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFV1 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFV2 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NDUFV3 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- NUBPL participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- OXA1L participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- PYURF participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- SFXN4 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- TIMMDC1 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- TMEM126A participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- TMEM126B participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only
- TMEM186 participates in Complex I biogenesisProteinReactome R-HSA-6799198Inferred only