Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

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

Gene

NDUFS6

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

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.

GO - Molecular functioni

  • electron carrier activity Source: UniProtKB
  • NADH dehydrogenase (ubiquinone) activity Source: UniProtKB

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

Electron transport, Respiratory chain, Transport

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.

Names & Taxonomyi

Protein namesi
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 namesi
Name:NDUFS6
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componenti: Chromosome 5

Organism-specific databases

HGNCiHGNC:7713. NDUFS6.

Subcellular locationi

GO - Cellular componenti

  • mitochondrial inner membrane Source: Reactome
  • mitochondrial respiratory chain complex I Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

Orphaneti2609. Isolated NADH-CoQ reductase deficiency.
PharmGKBiPA31523.

Polymorphism and mutation databases

BioMutaiNDUFS6.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 2828MitochondrionBy similarityAdd
BLAST
Chaini29 – 12496NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrialPRO_0000020020Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei98 – 981N6-acetyllysineBy similarity

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiO75380.
PaxDbiO75380.
PeptideAtlasiO75380.
PRIDEiO75380.

2D gel databases

UCD-2DPAGEO75380.

PTM databases

PhosphoSiteiO75380.

Expressioni

Gene expression databases

BgeeiO75380.
CleanExiHS_NDUFS6.
ExpressionAtlasiO75380. baseline and differential.
GenevisibleiO75380. HS.

Organism-specific databases

HPAiHPA064221.

Interactioni

Subunit structurei

Mammalian complex I is composed of 45 different subunits. This is a component of the iron-sulfur (IP) fragment of the enzyme.1 Publication

Protein-protein interaction databases

BioGridi110805. 41 interactions.
IntActiO75380. 7 interactions.
MINTiMINT-1427509.
STRINGi9606.ENSP00000274137.

Structurei

3D structure databases

ProteinModelPortaliO75380.
SMRiO75380. Positions 59-119.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I NDUFS6 subunit family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiNOG81252.
HOGENOMiHOG000161276.
HOVERGENiHBG006549.
InParanoidiO75380.
KOiK03939.
OMAiTHTGQAW.
OrthoDBiEOG7W41F0.
PhylomeDBiO75380.
TreeFamiTF315128.

Family and domain databases

Gene3Di2.60.260.40. 1 hit.
InterProiIPR016668. NADH_Ub_cplx-1_Fe-S_6_mit.
IPR019401. Znf_CHCC.
[Graphical view]
PfamiPF10276. zf-CHCC. 1 hit.
[Graphical view]
PIRSFiPIRSF016564. CI-13KD-A. 1 hit.

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

O75380-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAAAMTFCRL LNRCGEAARS LPLGARCFGV RVSPTGEKVT HTGQVYDDKD
60 70 80 90 100
YRRIRFVGRQ KEVNENFAID LIAEQPVSEV ETRVIACDGG GGALGHPKVY
110 120
INLDKETKTG TCGYCGLQFR QHHH
Length:124
Mass (Da):13,712
Last modified:November 1, 1998 - v1
Checksum:i0A1465160BCA772D
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF044959 mRNA. Translation: AAC27799.1.
BC038664 mRNA. Translation: AAH38664.1.
BC046155 mRNA. Translation: AAH46155.1.
CCDSiCCDS3866.1.
PIRiJE0194.
RefSeqiNP_004544.1. NM_004553.4.
UniGeneiHs.408257.

Genome annotation databases

EnsembliENST00000274137; ENSP00000274137; ENSG00000145494.
GeneIDi4726.
KEGGihsa:4726.
UCSCiuc003jcy.3. human.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF044959 mRNA. Translation: AAC27799.1.
BC038664 mRNA. Translation: AAH38664.1.
BC046155 mRNA. Translation: AAH46155.1.
CCDSiCCDS3866.1.
PIRiJE0194.
RefSeqiNP_004544.1. NM_004553.4.
UniGeneiHs.408257.

3D structure databases

ProteinModelPortaliO75380.
SMRiO75380. Positions 59-119.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110805. 41 interactions.
IntActiO75380. 7 interactions.
MINTiMINT-1427509.
STRINGi9606.ENSP00000274137.

Chemistry

ChEMBLiCHEMBL2363065.

PTM databases

PhosphoSiteiO75380.

Polymorphism and mutation databases

BioMutaiNDUFS6.

2D gel databases

UCD-2DPAGEO75380.

Proteomic databases

MaxQBiO75380.
PaxDbiO75380.
PeptideAtlasiO75380.
PRIDEiO75380.

Protocols and materials databases

DNASUi4726.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000274137; ENSP00000274137; ENSG00000145494.
GeneIDi4726.
KEGGihsa:4726.
UCSCiuc003jcy.3. human.

Organism-specific databases

CTDi4726.
GeneCardsiGC05P001801.
HGNCiHGNC:7713. NDUFS6.
HPAiHPA064221.
MIMi603848. gene.
neXtProtiNX_O75380.
Orphaneti2609. Isolated NADH-CoQ reductase deficiency.
PharmGKBiPA31523.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiNOG81252.
HOGENOMiHOG000161276.
HOVERGENiHBG006549.
InParanoidiO75380.
KOiK03939.
OMAiTHTGQAW.
OrthoDBiEOG7W41F0.
PhylomeDBiO75380.
TreeFamiTF315128.

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.

Miscellaneous databases

ChiTaRSiNDUFS6. human.
GeneWikiiNDUFS6.
GenomeRNAii4726.
NextBioi18226.
PROiO75380.
SOURCEiSearch...

Gene expression databases

BgeeiO75380.
CleanExiHS_NDUFS6.
ExpressionAtlasiO75380. baseline and differential.
GenevisibleiO75380. HS.

Family and domain databases

Gene3Di2.60.260.40. 1 hit.
InterProiIPR016668. NADH_Ub_cplx-1_Fe-S_6_mit.
IPR019401. Znf_CHCC.
[Graphical view]
PfamiPF10276. zf-CHCC. 1 hit.
[Graphical view]
PIRSFiPIRSF016564. CI-13KD-A. 1 hit.
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  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: IDENTIFICATION IN THE NADH-UBIQUINONE OXIDOREDUCTASE COMPLEX, IDENTIFICATION BY MASS SPECTROMETRY.
  4. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  5. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
    Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.
    J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

Entry informationi

Entry nameiNDUS6_HUMAN
AccessioniPrimary (citable) accession number: O75380
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 15, 1998
Last sequence update: November 1, 1998
Last modified: June 24, 2015
This is version 122 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny 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.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 5
    Human chromosome 5: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.