O00483 (NDUA4_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4

Alternative name(s):
Complex I-MLRQ
Short name=CI-MLRQ
NADH-ubiquinone oxidoreductase MLRQ subunit

Gene names

Name:

NDUFA4

Organism

Homo sapiens (Human)

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

Protein attributes

Sequence length	81 AA.
Sequence status	Complete.
Protein existence	Evidence at protein level

General annotation (Comments)

Function	Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed to be not involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
Subunit structure	Mammalian complex I is composed of 45 different subunits.
Subcellular location	Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
Sequence similarities	Belongs to the complex I NDUFA4 subunit family.

Ontologies

Keywords
Biological process	Electron transport Respiratory chain Transport
Cellular component	Membrane Mitochondrion Mitochondrion inner membrane
PTM	Acetylation
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological process	mitochondrial electron transport, NADH to ubiquinone Non-traceable author statement. Source: UniProtKB transport Inferred from electronic annotation. Source: UniProtKB-KW
Cellular component	mitochondrial respiratory chain complex I Inferred from direct assay Ref.8. Source: UniProtKB
Molecular function	NADH dehydrogenase (ubiquinone) activity Non-traceable author statement. Source: UniProtKB
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Chain	1 – 81	81	NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4		PRO_0000118821
Amino acid modifications
	Modified residue	55	1	N6-acetyllysine By similarity

Sequences

	Sequence		Length	Mass (Da)
	O00483 [UniParc]. Last modified July 1, 1997. Version 1. Checksum: 2FA1D1115EDE24C7	FASTA	81	9,370
	10 20 30 40 50 60 MLRQIIGQAK KHPSLIPLFV FIGTGATGAT LYLLRLALFN PDVCWDRNNP EPWNKLGPND 70 80 QYKFYSVNVD YSKLKKERPD F

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Cloning of the human cDNA sequence encoding the NADH:ubiquinone oxidoreductase MLRQ subunit." Kim J.W., Lee Y., Kang H.B., Chose Y.K., Chung T.W., Chang S.Y., Lee K.S., Choe I.S. Biochem. Mol. Biol. Int. 43:669-675(1997) [PubMed: 9352085] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA]. Tissue: Liver.
[2]	"Genomic sequence, cDNA sequence and chromosomal localization of the NDUFA4 human gene coding for the MLRQ subunit of NADH:ubiquinone oxidoreductase and its pseudogene." Kanagarajah D., Raha S., Scherer S., Robinson B.H. Submitted (NOV-1999) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[3]	"Cloning of human full open reading frames in Gateway(TM) system entry vector (pDONR201)." Ebert L., Schick M., Neubert P., Schatten R., Henze S., Korn B. Submitted (MAY-2004) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
[4]	"Cloning of human full open reading frames in Gateway(TM) system entry vector (pDONR201)." Halleck A., Ebert L., Mkoundinya M., Schick M., Eisenstein S., Neubert P., Kstrang K., Schatten R., Shen B., Henze S., Mar W., Korn B., Zuo D., Hu Y., LaBaer J. Submitted (JUN-2004) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
[5]	"Human chromosome 7: DNA sequence and biology." Scherer S.W., Cheung J., MacDonald J.R., Osborne L.R., Nakabayashi K., Herbrick J.-A., Carson A.R., Parker-Katiraee L., Skaug J., Khaja R., Zhang J., Hudek A.K., Li M., Haddad M., Duggan G.E., Fernandez B.A., Kanematsu E., Gentles S. , Christopoulos C.C., Choufani S., Kwasnicka D., Zheng X.H., Lai Z., Nusskern D.R., Zhang Q., Gu Z., Lu F., Zeesman S., Nowaczyk M.J., Teshima I., Chitayat D., Shuman C., Weksberg R., Zackai E.H., Grebe T.A., Cox S.R., Kirkpatrick S.J., Rahman N., Friedman J.M., Heng H.H.Q., Pelicci P.G., Lo-Coco F., Belloni E., Shaffer L.G., Pober B., Morton C.C., Gusella J.F., Bruns G.A.P., Korf B.R., Quade B.J., Ligon A.H., Ferguson H., Higgins A.W., Leach N.T., Herrick S.R., Lemyre E., Farra C.G., Kim H.-G., Summers A.M., Gripp K.W., Roberts W., Szatmari P., Winsor E.J.T., Grzeschik K.-H., Teebi A., Minassian B.A., Kere J., Armengol L., Pujana M.A., Estivill X., Wilson M.D., Koop B.F., Tosi S., Moore G.E., Boright A.P., Zlotorynski E., Kerem B., Kroisel P.M., Petek E., Oscier D.G., Mould S.J., Doehner H., Doehner K., Rommens J.M., Vincent J.B., Venter J.C., Li P.W., Mural R.J., Adams M.D., Tsui L.-C. Science 300:767-772(2003) [PubMed: 12690205] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[6]	Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C. Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[7]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Brain.
[8]	"The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification." Murray J., Zhang B., Taylor S.W., Oglesbee D., Fahy E., Marusich M.F., Ghosh S.S., Capaldi R.A. J. Biol. Chem. 278:13619-13622(2003) [PubMed: 12611891] [Abstract] Cited for: MASS SPECTROMETRY, IDENTIFICATION IN THE NADH-UBIQUINONE OXIDOREDUCTASE COMPLEX.
[9]	"Initial characterization of the human central proteome." Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J. BMC Syst. Biol. 5:17-17(2011) [PubMed: 21269460] [Abstract] Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	U94586 mRNA. Translation: AAB52726.1. AF201077 mRNA. Translation: AAF09253.1. CR407618 mRNA. Translation: CAG28546.1. CR541716 mRNA. Translation: CAG46517.1. CH236948 Genomic DNA. Translation: EAL24297.1. CH471073 Genomic DNA. Translation: EAW93631.1. BC105295 mRNA. Translation: AAI05296.1. BC101794 mRNA. Translation: AAI01795.1. BC101796 mRNA. Translation: AAI01797.1.
IPI	IPI00011770.
RefSeq	NP_002480.1. NM_002489.3.
UniGene	Hs.50098.
3D structure databases
ProteinModelPortal	O00483.
ModBase	Search...
Protein-protein interaction databases
IntAct	O00483. 4 interactions.
STRING	O00483.
PTM databases
PhosphoSite	O00483.
Proteomic databases
PeptideAtlas	O00483.
PRIDE	O00483.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000339600; ENSP00000339720; ENSG00000189043.
GeneID	4697.
KEGG	hsa:4697.
UCSC	uc003srx.1. human.
Organism-specific databases
CTD	4697.
GeneCards	GC07M010938.
H-InvDB	HIX0006484.
HGNC	HGNC:7687. NDUFA4.
MIM	603833. gene.
neXtProt	NX_O00483.
PharmGKB	PA31493.
GenAtlas	Search...
Phylogenomic databases
eggNOG	prNOG21183.
GeneTree	ENSGT00390000010457.
HOGENOM	HBG738659.
HOVERGEN	HBG006548.
InParanoid	O00483.
OMA	YKFFAVN.
OrthoDB	EOG4TF0MX.
PhylomeDB	O00483.
Enzyme and pathway databases
Reactome	REACT_111217. Metabolism.
Gene expression databases
ArrayExpress	O00483.
Bgee	O00483.
CleanEx	HS_NDUFA4.
Genevestigator	O00483.
GermOnline	ENSG00000189043. Homo sapiens.
Family and domain databases
InterPro	IPR010530. B12D. [Graphical view]
KO	K03948.
PANTHER	PTHR14256. PTHR14256. 1 hit.
Pfam	PF06522. B12D. 1 hit. [Graphical view]
ProtoNet	Search...
Other
DrugBank	DB00157. NADH.
NextBio	18114.
SOURCE	Search...

Entry information

Entry name

NDUA4_HUMAN

Accession

Primary (citable) accession number: O00483
Secondary accession number(s): A4D109, Q6FHN5

Entry history

Integrated into UniProtKB/Swiss-Prot:	November 1, 1997
Last sequence update:	July 1, 1997
Last modified:	January 25, 2012
This is version 95 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.