NDUA4_BOVIN
ID NDUA4_BOVIN Reviewed; 82 AA.
AC Q01321; Q3ZC70;
DT 01-JUL-1993, integrated into UniProtKB/Swiss-Prot.
DT 01-JUL-1993, sequence version 1.
DT 03-AUG-2022, entry version 132.
DE RecName: Full=Cytochrome c oxidase subunit NDUFA4;
DE AltName: Full=Complex I-MLRQ;
DE Short=CI-MLRQ;
DE AltName: Full=NADH-ubiquinone oxidoreductase MLRQ subunit;
GN Name=NDUFA4;
OS Bos taurus (Bovine).
OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC Eutheria; Laurasiatheria; Artiodactyla; Ruminantia; Pecora; Bovidae;
OC Bovinae; Bos.
OX NCBI_TaxID=9913;
RN [1]
RP NUCLEOTIDE SEQUENCE [MRNA], AND PROTEIN SEQUENCE OF 1-39 AND 49-74.
RC TISSUE=Heart;
RX PubMed=1518044; DOI=10.1016/0022-2836(92)91052-q;
RA Walker J.E., Arizmendi J.M., Dupuis A., Fearnley I.M., Finel M., Medd S.M.,
RA Pilkington S.J., Runswick M.J., Skehel J.M.;
RT "Sequences of 20 subunits of NADH:ubiquinone oxidoreductase from bovine
RT heart mitochondria. Application of a novel strategy for sequencing proteins
RT using the polymerase chain reaction.";
RL J. Mol. Biol. 226:1051-1072(1992).
RN [2]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC STRAIN=Crossbred X Angus; TISSUE=Ileum;
RG NIH - Mammalian Gene Collection (MGC) project;
RL Submitted (AUG-2005) to the EMBL/GenBank/DDBJ databases.
RN [3]
RP MASS SPECTROMETRY, AND INITIAL IDENTIFICATION AS SUBUNIT OF THE
RP NADH-UBIQUINONE OXIDOREDUCTASE COMPLEX.
RX PubMed=12837546; DOI=10.1016/s0005-2728(03)00059-8;
RA Hirst J., Carroll J., Fearnley I.M., Shannon R.J., Walker J.E.;
RT "The nuclear encoded subunits of complex I from bovine heart
RT mitochondria.";
RL Biochim. Biophys. Acta 1604:135-150(2003).
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 (By similarity). NDUFA4 is
CC required for complex IV maintenance (By similarity).
CC {ECO:0000250|UniProtKB:O00483, ECO:0000250|UniProtKB:P00401}.
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 COX4I1 (or
CC COX4I2), COX5A, COX5B, COX6A2 (or COX6A1), COX6B1 (or COX6B2), COX6C,
CC COX7A1 (or COX7A2), COX7B, COX7C, COX8B and NDUFA4, which are encoded
CC in the nuclear genome. The complex exists as a monomer or a dimer and
CC forms supercomplexes (SCs) in the inner mitochondrial membrane with
CC NADH-ubiquinone oxidoreductase (complex I, CI) and ubiquinol-cytochrome
CC c oxidoreductase (cytochrome b-c1 complex, complex III, CIII),
CC resulting in different assemblies (supercomplex SCI(1)III(2)IV(1) and
CC megacomplex MCI(2)III(2)IV(2)) (By similarity). Interacts with RAB5IF
CC (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}.
CC -!- CAUTION: Was initially believed to be a subunit of the mitochondrial
CC membrane respiratory chain NADH dehydrogenase (complex I). However, was
CC not present in all complex I preparations and presence correlated with
CC contamination by COX6B1 (PubMed:12837546).
CC {ECO:0000305|PubMed:12837546}.
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DR EMBL; X64897; CAA46106.1; -; mRNA.
DR EMBL; BC102882; AAI02883.1; -; mRNA.
DR PIR; S28255; S28255.
DR RefSeq; NP_787014.1; NM_175820.3.
DR AlphaFoldDB; Q01321; -.
DR SMR; Q01321; -.
DR CORUM; Q01321; -.
DR IntAct; Q01321; 2.
DR STRING; 9913.ENSBTAP00000014801; -.
DR TCDB; 3.D.1.6.1; the h(+) or na(+)-translocating nadh dehydrogenase (ndh) family.
DR PaxDb; Q01321; -.
DR PeptideAtlas; Q01321; -.
DR PRIDE; Q01321; -.
DR Ensembl; ENSBTAT00000014801; ENSBTAP00000014801; ENSBTAG00000011145.
DR GeneID; 327704; -.
DR KEGG; bta:327704; -.
DR CTD; 4697; -.
DR VEuPathDB; HostDB:ENSBTAG00000011145; -.
DR VGNC; VGNC:31947; NDUFA4.
DR eggNOG; ENOG502S65P; Eukaryota.
DR GeneTree; ENSGT00940000154268; -.
DR HOGENOM; CLU_181002_0_0_1; -.
DR InParanoid; Q01321; -.
DR OMA; QHKFYSP; -.
DR OrthoDB; 1552648at2759; -.
DR TreeFam; TF106383; -.
DR Reactome; R-BTA-5628897; TP53 Regulates Metabolic Genes.
DR Reactome; R-BTA-611105; Respiratory electron transport.
DR Reactome; R-BTA-9707564; Cytoprotection by HMOX1.
DR Proteomes; UP000009136; Chromosome 4.
DR Bgee; ENSBTAG00000011145; Expressed in cardiac ventricle and 105 other tissues.
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 1: Evidence at protein level;
KW Acetylation; Direct protein sequencing; Electron transport; Membrane;
KW Mitochondrion; Mitochondrion inner membrane; Phosphoprotein;
KW Reference proteome; Respiratory chain; Transmembrane; Transmembrane helix;
KW Transport.
FT CHAIN 1..82
FT /note="Cytochrome c oxidase subunit NDUFA4"
FT /id="PRO_0000118820"
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..82
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 67
FT /note="Phosphoserine"
FT /evidence="ECO:0000250|UniProtKB:O00483"
SQ SEQUENCE 82 AA; 9325 MW; 3F9D1D228CD4E907 CRC64;
MLRQIIGQAK RHPSLIPLFI FIGAGGTGAA LYVTRLALFN PDVSWDRKNN PEPWNKLGPN
DQYKFYSVNV DYSKLKKEGP DF