O75380 (NDUS6_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial

Alternative name(s):
Complex I-13kD-A
Short name=CI-13kD-A
NADH-ubiquinone oxidoreductase 13 kDa-A subunit

Gene names

Name:

NDUFS6

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	124 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	Mammalian complex I is composed of 45 different subunits. This is a component of the iron-sulfur (IP) fragment of the enzyme. Ref.3
Subcellular location	Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
Sequence similarities	Belongs to the complex I NDUFS6 subunit family.

Ontologies

Keywords
Biological process	Electron transport Respiratory chain Transport
Cellular component	Membrane Mitochondrion Mitochondrion inner membrane
Domain	Transit peptide
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	mitochondrial electron transport, NADH to ubiquinone Non-traceable author statement Ref.1PubMed 9878551. Source: UniProtKB small molecule metabolic process Traceable author statement. Source: Reactome
Cellular_component	mitochondrial respiratory chain complex I Inferred from direct assay Ref.3. Source: UniProtKB
Molecular_function	NADH dehydrogenase (ubiquinone) activity Non-traceable author statement Ref.1PubMed 9878551. Source: UniProtKB electron carrier activity Non-traceable author statement Ref.1. Source: UniProtKB
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 28	28	Mitochondrion By similarity
	Chain	29 – 124	96	NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial		PRO_0000020020

Sequences

	Sequence		Length	Mass (Da)
	O75380 [UniParc]. Last modified November 1, 1998. Version 1. Checksum: 0A1465160BCA772D	FASTA	124	13,712
	10 20 30 40 50 60 MAAAMTFCRL LNRCGEAARS LPLGARCFGV RVSPTGEKVT HTGQVYDDKD YRRIRFVGRQ 70 80 90 100 110 120 KEVNENFAID LIAEQPVSEV ETRVIACDGG GGALGHPKVY INLDKETKTG TCGYCGLQFR QHHH

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"cDNA sequence and chromosomal localization of the remaining three human nuclear encoded iron sulphur protein (IP) subunits of complex I: the human IP fraction is completed." Loeffen J., van den Heuvel L., Smeets R., Triepels R., Sengers R., Trijbels F., Smeitink J. Biochem. Biophys. Res. Commun. 247:751-758(1998) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[2]	"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 and Skin.
[3]	"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.
[4]	"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	AF044959 mRNA. Translation: AAC27799.1. BC038664 mRNA. Translation: AAH38664.1. BC046155 mRNA. Translation: AAH46155.1.
IPI	IPI00025344.
PIR	JE0194.
RefSeq	NP_004544.1. NM_004553.4.
UniGene	Hs.408257.
3D structure databases
ProteinModelPortal	O75380.
ModBase	Search...
Protein-protein interaction databases
IntAct	O75380. 3 interactions.
MINT	MINT-1427509.
STRING	9606.ENSP00000274137.
PTM databases
PhosphoSite	O75380.
2D gel databases
UCD-2DPAGE	O75380.
Proteomic databases
PaxDb	O75380.
PeptideAtlas	O75380.
PRIDE	O75380.
Protocols and materials databases
DNASU	4726.
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000274137; ENSP00000274137; ENSG00000145494.
GeneID	4726.
KEGG	hsa:4726.
UCSC	uc003jcy.3. human.
Organism-specific databases
CTD	4726.
GeneCards	GC05P001801.
HGNC	HGNC:7713. NDUFS6.
MIM	603848. gene.
neXtProt	NX_O75380.
Orphanet	2609. Isolated NADH-CoQ reductase deficiency.
PharmGKB	PA31523.
GenAtlas	Search...
Phylogenomic databases
eggNOG	NOG81252.
HOGENOM	HOG000161276.
HOVERGEN	HBG006549.
InParanoid	O75380.
KO	K03939.
OMA	THTGQAW.
OrthoDB	EOG4THVVK.
PhylomeDB	O75380.
Enzyme and pathway databases
Reactome	REACT_111217. Metabolism.
Gene expression databases
ArrayExpress	O75380.
Bgee	O75380.
CleanEx	HS_NDUFS6.
Genevestigator	O75380.
GermOnline	ENSG00000145494. Homo sapiens.
Family and domain databases
Gene3D	2.60.260.40. 1 hit.
InterPro	IPR016668. NADH_Ub_cplx-1_Fe-S_6_mit. IPR019401. Znf_CHCC. [Graphical view]
Pfam	PF10276. zf-CHCC. 1 hit. [Graphical view]
PIRSF	PIRSF016564. CI-13KD-A. 1 hit.
ProtoNet	Search...
Other
ChiTaRS	NDUFS6. human.
DrugBank	DB00157. NADH.
GenomeRNAi	4726.
NextBio	18226.
SOURCE	Search...

Entry information

Entry name

NDUS6_HUMAN

Accession

Primary (citable) accession number: O75380

Entry history

Integrated into UniProtKB/Swiss-Prot:	December 15, 1998
Last sequence update:	November 1, 1998
Last modified:	May 1, 2013
This is version 105 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.