Pathway
Complex IV assembly
At least 30 proteins are required to form the functional human complex IV, 14 of which are complex subunits. The complex contains the cofactors heme a, heme a3, and one mononuclear copper (CuB) center in the COX1 subunit, and a binuclear copper (CuA) in COX2, as well as several lipid molecules (phosphatidylethanolamine, PE, cardiolipin, CL) in different subunits (PDB 5Z62; Zong et al., 2018). The insertion of these cofactors is an intricate process requiring many assembly factors (Nývltová et al, 2022; reviewed in Mick et al., 2011; Timón-Gómez et al., 2018; Watson & McStay, 2020; Dennerlein et al., 2023).
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Mutations in any complex IV subunits or assembly factors lead to different types of complex IV deficiency, usually manifesting as Leigh syndrome (MIM:220110; reviewed in Pecina et al., 2004; Čunátová et al., 2020)
REACT:R-HSA-9864848Reactome
What the reference databases state
Statements
44 statements
44 statements · 1 reference database · Subject: Complex IV assembly · Sources: Reactome
- COA1 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COA3 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COA5 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COQ10A participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COQ10B participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX11 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX14 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX15 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX16 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX17 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX19 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX20 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
Show the remaining 32
- COX4I1 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX4I2 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX5A participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX5B participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX6A1 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX6A2 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX6B1 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX6B2 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX6C participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX7A1 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX7A2 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX7A2L participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX7B participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX7C participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COX8A participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- COXFA4 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- HIGD1A participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- HIGD2A participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- MT-CO1 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- MT-CO2 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- MT-CO3 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- PET100 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- PET117 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- RAB5IF participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- SCO1 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- SCO2 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- SMIM20 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- SURF1 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- TACO1 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- TIMM21 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- TMEM177 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only
- TMEM223 participates in Complex IV assemblyProteinReactome R-HSA-9864848Inferred only