O43676 (NDUB3_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3

Alternative name(s):
Complex I-B12
Short name=CI-B12
NADH-ubiquinone oxidoreductase B12 subunit

Gene names

Name:

NDUFB3

Organism

Homo sapiens (Human) [Reference proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo

Protein attributes

Sequence length	98 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
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 not to be 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	Complex I is composed of 45 different subunits. Ref.7
Subcellular location	Mitochondrion inner membrane; Single-pass membrane protein; Matrix side.
Sequence similarities	Belongs to the complex I NDUFB3 subunit family.

Ontologies

Keywords
Biological process	Electron transport Respiratory chain Transport
Cellular component	Membrane Mitochondrion Mitochondrion inner membrane
Domain	Transmembrane Transmembrane helix
PTM	Acetylation Methylation
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	mitochondrial electron transport, NADH to ubiquinone Non-traceable author statement Ref.2. Source: UniProtKB small molecule metabolic process Traceable author statement. Source: Reactome
Cellular_component	integral to membrane Inferred from electronic annotation. Source: UniProtKB-KW mitochondrial respiratory chain complex I Inferred from direct assay Ref.7. Source: UniProtKB
Molecular_function	NADH dehydrogenase (ubiquinone) activity Non-traceable author statement Ref.2. Source: UniProtKB
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Initiator methionine	1	1	Removed By similarity
	Chain	2 – 98	97	NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3		PRO_0000118797
Regions
	Transmembrane	66 – 88	23	Helical; Potential
Amino acid modifications
	Modified residue	2	1	N-acetylalanine By similarity
	Modified residue	5	1	Methylhistidine By similarity
	Modified residue	7	1	Methylhistidine By similarity
	Modified residue	9	1	Methylhistidine By similarity
	Modified residue	23	1	N6-acetyllysine By similarity
	Modified residue	34	1	N6-acetyllysine By similarity

Sequences

	Sequence		Length	Mass (Da)
	O43676 [UniParc]. Last modified January 23, 2007. Version 3. Checksum: 1C77F0C7A4DC757F	FASTA	98	11,402
	10 20 30 40 50 60 MAHEHGHEHG HHKMELPDYR QWKIEGTPLE TIQKKLAAKG LRDPWGRNEA WRYMGGFAKS 70 80 90 VSFSDVFFKG FKWGFAAFVV AVGAEYYLES LNKDKKHH

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Identification and primary structure of five human NADH-ubiquinone oxidoreductase subunits." Ton C., Hwang D.M., Dempsey A.A., Liew C.-C. Biochem. Biophys. Res. Commun. 241:589-594(1997) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA]. Tissue: Heart.
[2]	"cDNA of eight nuclear encoded subunits of NADH:ubiquinone oxidoreductase: human complex I cDNA characterization completed." Loeffen J.L.C.M., Triepels R.H., van den Heuvel L.P., Schuelke M., Buskens C.A.F., Smeets R.J.P., Trijbels J.M.F., Smeitink J.A.M. Biochem. Biophys. Res. Commun. 253:415-422(1998) [PubMed] [Europe PMC] [Abstract] 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 (JUN-2004) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
[4]	"Generation and annotation of the DNA sequences of human chromosomes 2 and 4." Hillier L.W., Graves T.A., Fulton R.S., Fulton L.A., Pepin K.H., Minx P., Wagner-McPherson C., Layman D., Wylie K., Sekhon M., Becker M.C., Fewell G.A., Delehaunty K.D., Miner T.L., Nash W.E., Kremitzki C., Oddy L., Du H. , Sun H., Bradshaw-Cordum H., Ali J., Carter J., Cordes M., Harris A., Isak A., van Brunt A., Nguyen C., Du F., Courtney L., Kalicki J., Ozersky P., Abbott S., Armstrong J., Belter E.A., Caruso L., Cedroni M., Cotton M., Davidson T., Desai A., Elliott G., Erb T., Fronick C., Gaige T., Haakenson W., Haglund K., Holmes A., Harkins R., Kim K., Kruchowski S.S., Strong C.M., Grewal N., Goyea E., Hou S., Levy A., Martinka S., Mead K., McLellan M.D., Meyer R., Randall-Maher J., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Shah N., Swearengen-Shahid S., Snider J., Strong J.T., Thompson J., Yoakum M., Leonard S., Pearman C., Trani L., Radionenko M., Waligorski J.E., Wang C., Rock S.M., Tin-Wollam A.-M., Maupin R., Latreille P., Wendl M.C., Yang S.-P., Pohl C., Wallis J.W., Spieth J., Bieri T.A., Berkowicz N., Nelson J.O., Osborne J., Ding L., Meyer R., Sabo A., Shotland Y., Sinha P., Wohldmann P.E., Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Jones T.A., She X., Ciccarelli F.D., Izaurralde E., Taylor J., Schmutz J., Myers R.M., Cox D.R., Huang X., McPherson J.D., Mardis E.R., Clifton S.W., Warren W.C., Chinwalla A.T., Eddy S.R., Marra M.A., Ovcharenko I., Furey T.S., Miller W., Eichler E.E., Bork P., Suyama M., Torrents D., Waterston R.H., Wilson R.K. Nature 434:724-731(2005) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[5]	Mural R.J., Istrail S., Sutton 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].
[6]	"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] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Lung.
[7]	"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] [Europe PMC] [Abstract] Cited for: MASS SPECTROMETRY, IDENTIFICATION IN THE NADH-UBIQUINONE OXIDOREDUCTASE COMPLEX.
[8]	"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] [Europe PMC] [Abstract] Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	AF047183 mRNA. Translation: AAC04268.1. AF035839 mRNA. Translation: AAC15590.1. CR456924 mRNA. Translation: CAG33205.1. AC007272 Genomic DNA. Translation: AAX88972.1. CH471063 Genomic DNA. Translation: EAW70233.1. BC018183 mRNA. Translation: AAH18183.1.
IPI	IPI00219383.
PIR	JC5822.
RefSeq	NP_001244031.1. NM_001257102.1. NP_002482.1. NM_002491.2.
UniGene	Hs.109760.
3D structure databases
ProteinModelPortal	O43676.
ModBase	Search...
Protein-protein interaction databases
IntAct	O43676. 2 interactions.
MINT	MINT-1416095.
STRING	9606.ENSP00000237889.
PTM databases
PhosphoSite	O43676.
Proteomic databases
PaxDb	O43676.
PRIDE	O43676.
Protocols and materials databases
DNASU	4709.
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000237889; ENSP00000237889; ENSG00000119013. ENST00000433898; ENSP00000410600; ENSG00000119013. ENST00000454214; ENSP00000407336; ENSG00000119013.
GeneID	4709.
KEGG	hsa:4709.
UCSC	uc002uwx.4. human.
Organism-specific databases
CTD	4709.
GeneCards	GC02P201936.
HGNC	HGNC:7698. NDUFB3.
HPA	HPA034596.
MIM	603839. gene.
neXtProt	NX_O43676.
Orphanet	2609. Isolated NADH-CoQ reductase deficiency.
PharmGKB	PA31504.
GenAtlas	Search...
Phylogenomic databases
eggNOG	NOG272434.
HOGENOM	HOG000173088.
HOVERGEN	HBG006448.
InParanoid	O43676.
KO	K03959.
OMA	LLKGFKW.
PhylomeDB	O43676.
Enzyme and pathway databases
Reactome	REACT_111217. Metabolism.
Gene expression databases
ArrayExpress	O43676.
Bgee	O43676.
CleanEx	HS_NDUFB3.
Genevestigator	O43676.
GermOnline	ENSG00000119013. Homo sapiens.
Family and domain databases
InterPro	IPR012576. NADH_UbQ_OxRdtase_B12_su. [Graphical view]
PANTHER	PTHR15082. PTHR15082. 1 hit.
Pfam	PF08122. NDUF_B12. 1 hit. [Graphical view]
ProtoNet	Search...
Other
DrugBank	DB00157. NADH.
GenomeRNAi	4709.
NextBio	18160.
SOURCE	Search...

Entry information

Entry name

NDUB3_HUMAN

Accession

Primary (citable) accession number: O43676
Secondary accession number(s): Q6IB80

Entry history

Integrated into UniProtKB/Swiss-Prot:	July 15, 1999
Last sequence update:	January 23, 2007
Last modified:	April 3, 2013
This is version 112 of the entry and version 3 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.