P56181 (NDUV3_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial

Alternative name(s):
Complex I-9kD
Short name=CI-9kD
NADH-ubiquinone oxidoreductase 9 kDa subunit
Renal carcinoma antigen NY-REN-4

Gene names

Name:

NDUFV3

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	108 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. This is a component of the flavoprotein-sulfur (FP) fragment of the enzyme.
Subcellular location	Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
Sequence similarities	Belongs to the complex I NDUFV3 subunit family.

Ontologies

Keywords
Biological process	Electron transport Respiratory chain Transport
Cellular component	Membrane Mitochondrion Mitochondrion inner membrane
Coding sequence diversity	Alternative splicing
Domain	Transit peptide
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 nucleus Inferred from direct assay. Source: HPA
Molecular function	NADH dehydrogenase (ubiquinone) activity Non-traceable author statement. Source: UniProtKB
Complete GO annotation...

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]

Isoform 1 (identifier: P56181-1)

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

Isoform 2 (identifier: P56181-2)

The sequence of this isoform differs from the canonical sequence as follows:
     56-57: KK → KNVVEPKERG...PGNDRGGTQE

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 34	34	Mitochondrion By similarity
	Chain	35 – 108	74	NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial		PRO_0000020025
Natural variations
	Alternative sequence	56 – 57	2	KK → KNVVEPKERGKLLATQTAAE LSKNLSSPSSYPPAVNKGRK VASPSPSGSVLFTDEGVPKF LSRKTLVEFPQKVLSPFRKQ GSDSEARQVGRKVTSPSSSS SSSSSDSESDDEADVSEVTP RVVSKGRGGLRKPEASHSFE NRAPRVTVSAKEKTLLQKPH VDITDPEKPHQPKKKGSPAK PSEGRENARPKTTMPRSQVD EEFLKQSLKEKQLQKTFRLN EIDKESQKPFEVKGPLPVHT KSGLSAPPKGSPAPAVLAEE ARAEGQLQASPPGAAEGHLE KPVPEPQRKAAPPLPRKETS GTQGIEGHLKGGQAIVEDQI PPSNLETVPVENNHGFHEKT AALKLEAEGEAMEDAAAPGN DRGGTQE in isoform 2.		VSP_038552
Experimental info
	Sequence conflict	9	1	Q → K in CAB56704. Ref.1
	Sequence conflict	57	1	K → SE Ref.1

Sequences

	Sequence		Length	Mass (Da)
	Isoform 1 [UniParc]. Last modified April 16, 2002. Version 2. Checksum: BD7375474AB0B1EC	FASTA	108	11,941
	10 20 30 40 50 60 MAAPCLLRQG RAGALKTMLQ EAQVFRGLAS TVSLSAESGK SEKGQPQNSK KQSPPKKPAP 70 80 90 100 VPAEPFDNTT YKNLQHHDYS TYTFLDLNLE LSKFRMPQPS SGRESPRH
	Isoform 2 [UniParc]. Checksum: BCC3F8EF45C0798A	FASTA	473	50,983
	10 20 30 40 50 60 MAAPCLLRQG RAGALKTMLQ EAQVFRGLAS TVSLSAESGK SEKGQPQNSK KQSPPKNVVE 70 80 90 100 110 120 PKERGKLLAT QTAAELSKNL SSPSSYPPAV NKGRKVASPS PSGSVLFTDE GVPKFLSRKT 130 140 150 160 170 180 LVEFPQKVLS PFRKQGSDSE ARQVGRKVTS PSSSSSSSSS DSESDDEADV SEVTPRVVSK 190 200 210 220 230 240 GRGGLRKPEA SHSFENRAPR VTVSAKEKTL LQKPHVDITD PEKPHQPKKK GSPAKPSEGR 250 260 270 280 290 300 ENARPKTTMP RSQVDEEFLK QSLKEKQLQK TFRLNEIDKE SQKPFEVKGP LPVHTKSGLS 310 320 330 340 350 360 APPKGSPAPA VLAEEARAEG QLQASPPGAA EGHLEKPVPE PQRKAAPPLP RKETSGTQGI 370 380 390 400 410 420 EGHLKGGQAI VEDQIPPSNL ETVPVENNHG FHEKTAALKL EAEGEAMEDA AAPGNDRGGT 430 440 450 460 470 QEPAPVPAEP FDNTTYKNLQ HHDYSTYTFL DLNLELSKFR MPQPSSGRES PRH

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Molecular cloning and characterization of the human mitochondrial NADH:oxidoreductase 10-kDa gene (NDUFV3)." de Coo R.F.M., Buddiger P., Smeets H.J.M., van Oost B.A. Genomics 45:434-437(1997) [PubMed: 9344673] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[2]	"Refined localization of autosomal recessive nonsyndromic deafness DFNB10 locus using 34 novel microsatellite markers, genomic structure, and exclusion of six known genes in the region." Berry A., Scott H.S., Kudoh J., Talior I., Korostishevsky M., Wattenhofer M., Guipponi M., Barras C., Rossier C., Shibuya K., Wang J., Kawasaki K., Asakawa S., Minoshima S., Shimizu N., Antonarakis S.E., Bonne-Tamir B. Genomics 68:22-29(2000) [PubMed: 10950923] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[3]	"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] (ISOFORM 1).
[4]	"Complete sequencing and characterization of 21,243 full-length human cDNAs." Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S. Nat. Genet. 36:40-45(2004) [PubMed: 14702039] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1). Tissue: Cerebellum.
[5]	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 (SEP-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: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2). Tissue: Brain and Uterus.
[7]	"Antigens recognized by autologous antibody in patients with renal-cell carcinoma." Scanlan M.J., Gordan J.D., Williamson B., Stockert E., Bander N.H., Jongeneel C.V., Gure A.O., Jaeger D., Jaeger E., Knuth A., Chen Y.-T., Old L.J. Int. J. Cancer 83:456-464(1999) [PubMed: 10508479] [Abstract] Cited for: IDENTIFICATION AS A RENAL CANCER ANTIGEN. Tissue: Renal cell carcinoma.
[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.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	X99726, X99727, X99728 Genomic DNA. Translation: CAB56704.1. Sequence problems. AB038163 Genomic DNA. Translation: BAB13732.1. CR542174 mRNA. Translation: CAG46971.1. CR542190 mRNA. Translation: CAG46987.1. AK289586 mRNA. Translation: BAF82275.1. CH471079 Genomic DNA. Translation: EAX09524.1. BC021217 mRNA. Translation: AAH21217.2. BC033766 mRNA. Translation: AAH33766.1. BC054016 mRNA. Translation: AAH54016.1.
IPI	IPI00008214. IPI00291016.
RefSeq	NP_001001503.1. NM_001001503.1. NP_066553.3. NM_021075.3.
UniGene	Hs.473937.
3D structure databases
ProteinModelPortal	P56181.
ModBase	Search...
Protein-protein interaction databases
IntAct	P56181. 2 interactions.
STRING	P56181.
PTM databases
PhosphoSite	P56181.
Proteomic databases
PRIDE	P56181.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000340344; ENSP00000342895; ENSG00000160194. ENST00000354250; ENSP00000346196; ENSG00000160194.
GeneID	4731.
KEGG	hsa:4731.
UCSC	uc002zcn.1. human.
Organism-specific databases
CTD	4731.
GeneCards	GC21P044299.
HGNC	HGNC:7719. NDUFV3.
HPA	CAB034142. HPA019791. HPA020463. HPA030427.
MIM	602184. gene.
neXtProt	NX_P56181.
PharmGKB	PA31529.
GenAtlas	Search...
Phylogenomic databases
eggNOG	prNOG13552.
GeneTree	ENSGT00390000012196.
HOVERGEN	HBG082013.
OrthoDB	EOG4894N4.
Enzyme and pathway databases
Pathway_Interaction_DB	hnf3apathway. FOXA1 transcription factor network.
Reactome	REACT_111217. Metabolism.
Gene expression databases
ArrayExpress	P56181.
Bgee	P56181.
CleanEx	HS_NDUFV3.
Genevestigator	P56181.
GermOnline	ENSG00000160194. Homo sapiens.
Family and domain databases
KO	K03944.
ProtoNet	Search...
Other
DrugBank	DB00157. NADH.
NextBio	18240.
SOURCE	Search...

Entry information

Entry name

NDUV3_HUMAN

Accession

Primary (citable) accession number: P56181
Secondary accession number(s): A8K0M1 , Q6FGD3, Q8WU60, Q9HCR5

Entry history

Integrated into UniProtKB/Swiss-Prot:	November 1, 1997
Last sequence update:	April 16, 2002
Last modified:	November 16, 2011
This is version 89 of the entry and version 2 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.