FB2024_03 , released June 25, 2024
Gene Group: MITOCHONDRIAL COMPLEX IV SUBUNITS
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General Information
Name
MITOCHONDRIAL COMPLEX IV SUBUNITS
Species
D. melanogaster
Symbol
MCIV
FlyBase ID
FBgg0000488
Date last reviewed
2021-02-26
Number of members
22
Description
Description
Cytochrome c oxidase (COX) is the terminal complex (complex IV) of the electron transfer chain, embedded in the mitochondrial inner membrane. It catalyzes electron transfer from reduced cytochrome c to molecular oxygen in a process coupled to the transfer of protons across the inner membrane, thus contributing to the generation of the proton gradient that is used by ATP synthase to drive ATP synthesis. Eukaryotic COX is a multimeric enzyme formed by subunits of dual genetic origin. The three subunits forming the catalytic core of the enzyme (COX1, COX2 and COX3) are encoded by the mitochondrial DNA. The remaining subunits are encoded by the nuclear DNA. The functions of these 'supernumerary subunits' are largely unknown, though are assumed to regulate complex IV activity and to coordinate energy production with cellular needs. (Adapted from PMID:17091399 and PMID:28870773.)
Notes on Group

NDUFA4 (aka ND-MLRQ) was originally assigned as a subunit of Complex I but is now known to be a supernumerary subunit of complex IV. (PMID:28870773)

Six supernumerary subunits of this complex are encoded by paralogous genes in D. melanogaster: COX4 and COX4L; COX5B and COX5BL; levy, COX6AL and COX6AL2; cype and COX6CL; COX7A, COX7AL and COX7AL2; COX7C and COX7CL. In each case, the first gene has widespread expression, whereas the other copy/copies (with an 'L' in the symbol) are expressed in a more restricted manner, usually specifically in the testis.

D. melanogaster COX7B (CG7630) is widely different in amino acid sequence, but similar in secondary and tertiary structures to mammalian COX7B, and is in fact replacing the latter subunit in virtually all protostome invertebrates (FBrf0254233).

Source Material
The MITOCHONDRIAL COMPLEX IV SUBUNITS Gene Group has been compiled using the following publication(s): Brischigliaro et al., 2022, Marygold, 2021.2.26, Rhooms et al., 2020, Porcelli and Marygold, 2014.9.16, Porcelli et al., 2007, Caggese et al., 2006, Tripoli et al., 2005, and Szuplewski and Terracol, 2001 .
Key Gene Ontology (GO) terms
Enzymatic activity
Enzyme name (EC)
cytochrome-c oxidase (7.1.1.9)
Related Gene Groups
Members (22)
For all members:
GO ribbon stack
 molecular functionbiological processcellular component
Gene Symbol
Gene Name
Also Known As
Source Material for Membership
Gene Symbol
Gene Name
Also Known As
Source Material for Membership
mitochondrial Cytochrome c oxidase subunit I
COI, COXI, COX1, cytochrome c oxidase subunit I, COX
mitochondrial Cytochrome c oxidase subunit II
COII, COXII, Cox2, COX, cytochrome c
mitochondrial Cytochrome c oxidase subunit III
COIII, COXIII, COX3, cytochrome c oxidase subunit III, COX
Gene Symbol
Gene Name
Also Known As
Source Material for Membership
Cytochrome c oxidase subunit 6A-like
Cytochrome c oxidase subunit 6A-like 2
Cytochrome c oxidase subunit 6C-like
Cytochrome c oxidase subunit 7A-like 2
Cytochrome c oxidase subunit 7B
Cytochrome c oxidase subunit 7C-like
NADH dehydrogenase (ubiquinone) MLRQ subunit
External Data
Other resource(s)

[Transporter Classification Database:The Proton-translocating Cytochrome Oxidase (COX) Superfamily](https://tcdb.org/search/result.php?tc=3.d.4)

Synonyms and Secondary IDs
Synonym(s)
Cytochrome A3
Respiratory chain complex IV
COX
MITOCHONDRIAL COMPLEX IV SUBUNITS
MITOCHONDRIAL COMPLEX IV - CYTOCHROME C OXIDASE SUBUNITS
MCIV
Secondary FlyBase ID(s)
References (10)