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Protein

NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6

Gene

NDUFB6

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

  • NADH dehydrogenase (ubiquinone) activity Source: UniProtKB

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

Electron transport, Respiratory chain, Transport

Enzyme and pathway databases

ReactomeiR-HSA-611105. Respiratory electron transport.
R-HSA-6799198. Complex I biogenesis.

Names & Taxonomyi

Protein namesi
Recommended name:
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6
Alternative name(s):
Complex I-B17
Short name:
CI-B17
NADH-ubiquinone oxidoreductase B17 subunit
Gene namesi
Name:NDUFB6
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 9

Organism-specific databases

HGNCiHGNC:7701. NDUFB6.

Subcellular locationi

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transmembranei68 – 8619HelicalSequence analysisAdd
BLAST

GO - Cellular componenti

  • integral component of membrane Source: UniProtKB-KW
  • mitochondrial inner membrane Source: Reactome
  • mitochondrial membrane Source: UniProtKB
  • mitochondrial respiratory chain complex I Source: UniProtKB
  • mitochondrion Source: HPA
  • nucleoplasm Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA31512.

Chemistry

ChEMBLiCHEMBL2363065.

Polymorphism and mutation databases

BioMutaiNDUFB6.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Initiator methionineiRemovedCombined sources
Chaini2 – 128127NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6PRO_0000118807Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei2 – 21N-acetylthreonineCombined sources
Modified residuei24 – 241N6-acetyllysineBy similarity

Keywords - PTMi

Acetylation

Proteomic databases

EPDiO95139.
MaxQBiO95139.
PaxDbiO95139.
PeptideAtlasiO95139.
PRIDEiO95139.
TopDownProteomicsiO95139-1. [O95139-1]
O95139-2. [O95139-2]

PTM databases

iPTMnetiO95139.
PhosphoSiteiO95139.

Expressioni

Gene expression databases

BgeeiO95139.
CleanExiHS_NDUFB6.
ExpressionAtlasiO95139. baseline and differential.
GenevisibleiO95139. HS.

Organism-specific databases

HPAiHPA044001.
HPA053334.

Interactioni

Subunit structurei

Complex I is composed of 45 different subunits.1 Publication

Protein-protein interaction databases

BioGridi110792. 27 interactions.
IntActiO95139. 8 interactions.
MINTiMINT-4981223.
STRINGi9606.ENSP00000369176.

Structurei

3D structure databases

ProteinModelPortaliO95139.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I NDUFB6 subunit family.Curated

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG4633. Eukaryota.
ENOG4111RT5. LUCA.
GeneTreeiENSGT00390000007535.
HOGENOMiHOG000231724.
HOVERGENiHBG006449.
InParanoidiO95139.
OMAiPAWIIHY.
OrthoDBiEOG7ZWD40.
PhylomeDBiO95139.
TreeFamiTF328587.

Family and domain databases

InterProiIPR019174. NADH_DH_b-subcmplx_su6.
[Graphical view]
PfamiPF09782. NDUF_B6. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: O95139-1) [UniParc]FASTAAdd to basket

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.

« Hide

        10         20         30         40         50
MTGYTPDEKL RLQQLRELRR RWLKDQELSP REPVLPPQKM GPMEKFWNKF
60 70 80 90 100
LENKSPWRKM VHGVYKKSIF VFTHVLVPVW IIHYYMKYHV SEKPYGIVEK
110 120
KSRIFPGDTI LETGEVIPPM KEFPDQHH
Length:128
Mass (Da):15,489
Last modified:January 23, 2007 - v3
Checksum:iE8F419F5EB154D0B
GO
Isoform 2 (identifier: O95139-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     92-106: Missing.

Note: No experimental confirmation available.
Show »
Length:113
Mass (Da):13,716
Checksum:iC5F9519567423E88
GO

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei92 – 10615Missing in isoform 2. 1 PublicationVSP_045233Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF035840 mRNA. Translation: AAC68838.1.
AF067167 mRNA. Translation: AAD32451.1.
CR456920 mRNA. Translation: CAG33201.1.
AK289702 mRNA. Translation: BAF82391.1.
AL353671 Genomic DNA. Translation: CAH71257.1.
AL353671 Genomic DNA. Translation: CAH71258.1.
CH471071 Genomic DNA. Translation: EAW58541.1.
CH471071 Genomic DNA. Translation: EAW58542.1.
BC009801 mRNA. Translation: AAH09801.1.
BC029247 mRNA. Translation: AAH29247.1.
BI669356 mRNA. No translation available.
CCDSiCCDS6528.1. [O95139-1]
CCDS6529.1. [O95139-2]
RefSeqiNP_002484.1. NM_002493.4. [O95139-1]
NP_877416.1. NM_182739.2. [O95139-2]
UniGeneiHs.493668.

Genome annotation databases

EnsembliENST00000350021; ENSP00000297983; ENSG00000165264. [O95139-2]
ENST00000379847; ENSP00000369176; ENSG00000165264. [O95139-1]
GeneIDi4712.
UCSCiuc003zre.3. human. [O95139-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF035840 mRNA. Translation: AAC68838.1.
AF067167 mRNA. Translation: AAD32451.1.
CR456920 mRNA. Translation: CAG33201.1.
AK289702 mRNA. Translation: BAF82391.1.
AL353671 Genomic DNA. Translation: CAH71257.1.
AL353671 Genomic DNA. Translation: CAH71258.1.
CH471071 Genomic DNA. Translation: EAW58541.1.
CH471071 Genomic DNA. Translation: EAW58542.1.
BC009801 mRNA. Translation: AAH09801.1.
BC029247 mRNA. Translation: AAH29247.1.
BI669356 mRNA. No translation available.
CCDSiCCDS6528.1. [O95139-1]
CCDS6529.1. [O95139-2]
RefSeqiNP_002484.1. NM_002493.4. [O95139-1]
NP_877416.1. NM_182739.2. [O95139-2]
UniGeneiHs.493668.

3D structure databases

ProteinModelPortaliO95139.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110792. 27 interactions.
IntActiO95139. 8 interactions.
MINTiMINT-4981223.
STRINGi9606.ENSP00000369176.

Chemistry

ChEMBLiCHEMBL2363065.

PTM databases

iPTMnetiO95139.
PhosphoSiteiO95139.

Polymorphism and mutation databases

BioMutaiNDUFB6.

Proteomic databases

EPDiO95139.
MaxQBiO95139.
PaxDbiO95139.
PeptideAtlasiO95139.
PRIDEiO95139.
TopDownProteomicsiO95139-1. [O95139-1]
O95139-2. [O95139-2]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000350021; ENSP00000297983; ENSG00000165264. [O95139-2]
ENST00000379847; ENSP00000369176; ENSG00000165264. [O95139-1]
GeneIDi4712.
UCSCiuc003zre.3. human. [O95139-1]

Organism-specific databases

CTDi4712.
GeneCardsiNDUFB6.
HGNCiHGNC:7701. NDUFB6.
HPAiHPA044001.
HPA053334.
MIMi603322. gene.
neXtProtiNX_O95139.
PharmGKBiPA31512.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4633. Eukaryota.
ENOG4111RT5. LUCA.
GeneTreeiENSGT00390000007535.
HOGENOMiHOG000231724.
HOVERGENiHBG006449.
InParanoidiO95139.
OMAiPAWIIHY.
OrthoDBiEOG7ZWD40.
PhylomeDBiO95139.
TreeFamiTF328587.

Enzyme and pathway databases

ReactomeiR-HSA-611105. Respiratory electron transport.
R-HSA-6799198. Complex I biogenesis.

Miscellaneous databases

GeneWikiiNDUFB6.
GenomeRNAii4712.
NextBioi18172.
PROiO95139.
SOURCEiSearch...

Gene expression databases

BgeeiO95139.
CleanExiHS_NDUFB6.
ExpressionAtlasiO95139. baseline and differential.
GenevisibleiO95139. HS.

Family and domain databases

InterProiIPR019174. NADH_DH_b-subcmplx_su6.
[Graphical view]
PfamiPF09782. NDUF_B6. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Molecular characterization and mutational analysis of the human B17 subunit of the mitochondrial respiratory chain complex I."
    Smeitink J., Loeffen J., Smeets R., Triepels R., Ruitenbeek W., Trijbels F., van den Heuvel L.
    Hum. Genet. 103:245-250(1998) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
  2. "Cloning and functional analysis of cDNAs with open reading frames for 300 previously undefined genes expressed in CD34+ hematopoietic stem/progenitor cells."
    Zhang Q.-H., Ye M., Wu X.-Y., Ren S.-X., Zhao M., Zhao C.-J., Fu G., Shen Y., Fan H.-Y., Lu G., Zhong M., Xu X.-R., Han Z.-G., Zhang J.-W., Tao J., Huang Q.-H., Zhou J., Hu G.-X.
    , Gu J., Chen S.-J., Chen Z.
    Genome Res. 10:1546-1560(2000) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
    Tissue: Umbilical cord blood.
  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] (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] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
    Tissue: Brain.
  5. "DNA sequence and analysis of human chromosome 9."
    Humphray S.J., Oliver K., Hunt A.R., Plumb R.W., Loveland J.E., Howe K.L., Andrews T.D., Searle S., Hunt S.E., Scott C.E., Jones M.C., Ainscough R., Almeida J.P., Ambrose K.D., Ashwell R.I.S., Babbage A.K., Babbage S., Bagguley C.L.
    , Bailey J., Banerjee R., Barker D.J., Barlow K.F., Bates K., Beasley H., Beasley O., Bird C.P., Bray-Allen S., Brown A.J., Brown J.Y., Burford D., Burrill W., Burton J., Carder C., Carter N.P., Chapman J.C., Chen Y., Clarke G., Clark S.Y., Clee C.M., Clegg S., Collier R.E., Corby N., Crosier M., Cummings A.T., Davies J., Dhami P., Dunn M., Dutta I., Dyer L.W., Earthrowl M.E., Faulkner L., Fleming C.J., Frankish A., Frankland J.A., French L., Fricker D.G., Garner P., Garnett J., Ghori J., Gilbert J.G.R., Glison C., Grafham D.V., Gribble S., Griffiths C., Griffiths-Jones S., Grocock R., Guy J., Hall R.E., Hammond S., Harley J.L., Harrison E.S.I., Hart E.A., Heath P.D., Henderson C.D., Hopkins B.L., Howard P.J., Howden P.J., Huckle E., Johnson C., Johnson D., Joy A.A., Kay M., Keenan S., Kershaw J.K., Kimberley A.M., King A., Knights A., Laird G.K., Langford C., Lawlor S., Leongamornlert D.A., Leversha M., Lloyd C., Lloyd D.M., Lovell J., Martin S., Mashreghi-Mohammadi M., Matthews L., McLaren S., McLay K.E., McMurray A., Milne S., Nickerson T., Nisbett J., Nordsiek G., Pearce A.V., Peck A.I., Porter K.M., Pandian R., Pelan S., Phillimore B., Povey S., Ramsey Y., Rand V., Scharfe M., Sehra H.K., Shownkeen R., Sims S.K., Skuce C.D., Smith M., Steward C.A., Swarbreck D., Sycamore N., Tester J., Thorpe A., Tracey A., Tromans A., Thomas D.W., Wall M., Wallis J.M., West A.P., Whitehead S.L., Willey D.L., Williams S.A., Wilming L., Wray P.W., Young L., Ashurst J.L., Coulson A., Blocker H., Durbin R.M., Sulston J.E., Hubbard T., Jackson M.J., Bentley D.R., Beck S., Rogers J., Dunham I.
    Nature 429:369-374(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  6. 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] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2).
    Tissue: Brain, Hypothalamus and Skin.
  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] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION IN THE NADH-UBIQUINONE OXIDOREDUCTASE COMPLEX, IDENTIFICATION BY MASS SPECTROMETRY.
  9. "Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach."
    Gauci S., Helbig A.O., Slijper M., Krijgsveld J., Heck A.J., Mohammed S.
    Anal. Chem. 81:4493-4501(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT THR-2, CLEAVAGE OF INITIATOR METHIONINE [LARGE SCALE ANALYSIS], IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  10. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  11. Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT THR-2, CLEAVAGE OF INITIATOR METHIONINE [LARGE SCALE ANALYSIS], IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

Entry informationi

Entry nameiNDUB6_HUMAN
AccessioniPrimary (citable) accession number: O95139
Secondary accession number(s): A8K0Y7, Q5VYT2, Q6IB84
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: January 23, 2007
Last modified: May 11, 2016
This is version 143 of the entry and version 3 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 9
    Human chromosome 9: 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

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 one 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.