NDUA4_PONPY
ID NDUA4_PONPY Reviewed; 81 AA.
AC Q0MQ97;
DT 03-OCT-2006, integrated into UniProtKB/Swiss-Prot.
DT 19-SEP-2006, sequence version 1.
DT 03-AUG-2022, entry version 62.
DE RecName: Full=Cytochrome c oxidase subunit NDUFA4;
GN Name=NDUFA4;
OS Pongo pygmaeus (Bornean orangutan).
OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae;
OC Pongo.
OX NCBI_TaxID=9600;
RN [1]
RP NUCLEOTIDE SEQUENCE [MRNA].
RX PubMed=16828987; DOI=10.1016/j.gene.2006.03.015;
RA Mishmar D., Ruiz-Pesini E., Mondragon-Palomino M., Procaccio V., Gaut B.,
RA Wallace D.C.;
RT "Adaptive selection of mitochondrial complex I subunits during primate
RT radiation.";
RL Gene 378:11-18(2006).
CC -!- FUNCTION: Component of the cytochrome c oxidase, the last enzyme in the
CC mitochondrial electron transport chain which drives oxidative
CC phosphorylation. The respiratory chain contains 3 multisubunit
CC complexes succinate dehydrogenase (complex II, CII), ubiquinol-
CC cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III,
CC CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to
CC transfer electrons derived from NADH and succinate to molecular oxygen,
CC creating an electrochemical gradient over the inner membrane that
CC drives transmembrane transport and the ATP synthase. Cytochrome c
CC oxidase is the component of the respiratory chain that catalyzes the
CC reduction of oxygen to water. Electrons originating from reduced
CC cytochrome c in the intermembrane space (IMS) are transferred via the
CC dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1
CC to the active site in subunit 1, a binuclear center (BNC) formed by
CC heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2
CC water molecules unsing 4 electrons from cytochrome c in the IMS and 4
CC protons from the mitochondrial matrix. NDUFA4 is required for complex
CC IV maintenance. {ECO:0000250|UniProtKB:O00483}.
CC -!- SUBUNIT: Component of the cytochrome c oxidase (complex IV, CIV), a
CC multisubunit enzyme composed of 14 subunits. The complex is composed of
CC a catalytic core of 3 subunits MT-CO1, MT-CO2 and MT-CO3, encoded in
CC the mitochondrial DNA, and 11 supernumerary subunits COX4I, COX5A,
CC COX5B, COX6A, COX6B, COX6C, COX7A, COX7B, COX7C, COX8 and NDUFA4, which
CC are encoded in the nuclear genome. The complex exists as a monomer or a
CC dimer and forms supercomplexes (SCs) in the inner mitochondrial
CC membrane with NADH-ubiquinone oxidoreductase (complex I, CI) and
CC ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex
CC III, CIII), resulting in different assemblies (supercomplex
CC SCI(1)III(2)IV(1) and megacomplex MCI(2)III(2)IV(2)) (By similarity).
CC Interacts with RAB5IF (By similarity). {ECO:0000250|UniProtKB:O00483}.
CC -!- SUBCELLULAR LOCATION: Mitochondrion inner membrane
CC {ECO:0000250|UniProtKB:O00483}; Single-pass membrane protein
CC {ECO:0000250|UniProtKB:O00483}.
CC -!- SIMILARITY: Belongs to the complex IV NDUFA4 subunit family.
CC {ECO:0000305}.
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DR EMBL; DQ885737; ABH12246.1; -; mRNA.
DR AlphaFoldDB; Q0MQ97; -.
DR SMR; Q0MQ97; -.
DR PRIDE; Q0MQ97; -.
DR GO; GO:0016021; C:integral component of membrane; IEA:UniProtKB-KW.
DR GO; GO:0005751; C:mitochondrial respiratory chain complex IV; ISS:UniProtKB.
DR GO; GO:1904960; P:positive regulation of cytochrome-c oxidase activity; ISS:UniProtKB.
DR InterPro; IPR010530; B12D.
DR PANTHER; PTHR14256; PTHR14256; 1.
DR Pfam; PF06522; B12D; 1.
PE 3: Inferred from homology;
KW Acetylation; Electron transport; Membrane; Mitochondrion;
KW Mitochondrion inner membrane; Phosphoprotein; Respiratory chain;
KW Transmembrane; Transmembrane helix; Transport.
FT CHAIN 1..81
FT /note="Cytochrome c oxidase subunit NDUFA4"
FT /id="PRO_0000251166"
FT TOPO_DOM 1..14
FT /note="Mitochondrial matrix"
FT /evidence="ECO:0000250|UniProtKB:O00483"
FT TRANSMEM 15..37
FT /note="Helical"
FT /evidence="ECO:0000250|UniProtKB:O00483"
FT TOPO_DOM 38..81
FT /note="Mitochondrial intermembrane"
FT /evidence="ECO:0000250|UniProtKB:O00483"
FT MOD_RES 10
FT /note="N6-acetyllysine"
FT /evidence="ECO:0000250|UniProtKB:Q62425"
FT MOD_RES 66
FT /note="Phosphoserine"
FT /evidence="ECO:0000250|UniProtKB:O00483"
SQ SEQUENCE 81 AA; 9386 MW; 5916C7404F9E7086 CRC64;
MLRQILSQAK KHPSLIPLFV FIGTGASGAT LYLLRLALFN PDVCWDRNNP EPWNKLGPND
QYKFYSVNVD YSKLKKERPD F