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Protein

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6

Gene

NDUFA6

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 to be not 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

ReactomeiREACT_22393. Respiratory electron transport.

Names & Taxonomyi

Protein namesi
Recommended name:
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6
Alternative name(s):
Complex I-B14
Short name:
CI-B14
LYR motif-containing protein 6
NADH-ubiquinone oxidoreductase B14 subunit
Gene namesi
Name:NDUFA6
Synonyms:LYRM6, NADHB14
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componentsi: Chromosome 22, Unplaced

Organism-specific databases

HGNCiHGNC:7690. NDUFA6.

Subcellular locationi

GO - Cellular componenti

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

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA31496.

Polymorphism and mutation databases

BioMutaiNDUFA6.
DMDMi298286909.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 154154NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6PRO_0000174302Add
BLAST

Proteomic databases

MaxQBiP56556.
PaxDbiP56556.
PRIDEiP56556.

PTM databases

PhosphoSiteiP56556.

Expressioni

Gene expression databases

BgeeiP56556.
CleanExiHS_NDUFA6.
ExpressionAtlasiP56556. baseline and differential.
GenevisibleiP56556. HS.

Organism-specific databases

HPAiHPA056934.

Interactioni

Subunit structurei

Mammalian complex I is composed of 45 different subunits.1 Publication

Protein-protein interaction databases

BioGridi110780. 4 interactions.
IntActiP56556. 5 interactions.
MINTiMINT-1381261.

Structurei

3D structure databases

ProteinModelPortaliP56556.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I LYR family.Curated

Phylogenomic databases

eggNOGiNOG283416.
GeneTreeiENSGT00390000018898.
HOGENOMiHOG000200993.
HOVERGENiHBG002032.
InParanoidiP56556.
KOiK03950.
OMAiWKQKGHI.
PhylomeDBiP56556.
TreeFamiTF105625.

Family and domain databases

InterProiIPR008011. Complex1_LYR.
[Graphical view]
PfamiPF05347. Complex1_LYR. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

P56556-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MGKDIRPRSA RAACKGVGLW SGCFGKMAGS GVRQATSTAS TFVKPIFSRD
60 70 80 90 100
MNEAKRRVRE LYRAWYREVP NTVHQFQLDI TVKMGRDKVR EMFMKNAHVT
110 120 130 140 150
DPRVVDLLVI KGKIELEETI KVWKQRTHVM RFFHETEAPR PKDFLSKFYV

GHDP
Length:154
Mass (Da):17,871
Last modified:June 15, 2010 - v3
Checksum:iC3423B7B75A6336A
GO

Sequence cautioni

The sequence AAC04267.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence BAF84563.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence BAG38151.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence EAW60489.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti32 – 321V → F in CAG32995 (Ref. 6) Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti35 – 351A → V.1 Publication
Corresponds to variant rs1801311 [ dbSNP | Ensembl ].
VAR_014483

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
CR456529 mRNA. Translation: CAG30415.1.
AL021878 Genomic DNA. No translation available.
CH471095 Genomic DNA. Translation: EAW60489.1. Different initiation.
AK291874 mRNA. Translation: BAF84563.1. Different initiation.
AK316562 mRNA. Translation: BAG38151.1. Different initiation.
AF047182 mRNA. Translation: AAC04267.1. Different initiation.
CR456714 mRNA. Translation: CAG32995.1.
CR541997 mRNA. Translation: CAG46794.1.
BC002772 mRNA. Translation: AAH02772.1.
CCDSiCCDS33656.1.
PIRiJC5821.
RefSeqiNP_002481.2. NM_002490.3.
UniGeneiHs.274416.

Genome annotation databases

EnsembliENST00000498737; ENSP00000418842; ENSG00000184983.
ENST00000605916; ENSP00000475402; ENSG00000272765.
ENST00000605927; ENSP00000475816; ENSG00000273397.
ENST00000610887; ENSP00000484723; ENSG00000277365.
ENST00000617763; ENSP00000482543; ENSG00000184983.
ENST00000627617; ENSP00000485713; ENSG00000281013.
ENST00000630201; ENSP00000487431; ENSG00000281013.
GeneIDi4700.
KEGGihsa:4700.
UCSCiuc003bcb.3. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
CR456529 mRNA. Translation: CAG30415.1.
AL021878 Genomic DNA. No translation available.
CH471095 Genomic DNA. Translation: EAW60489.1. Different initiation.
AK291874 mRNA. Translation: BAF84563.1. Different initiation.
AK316562 mRNA. Translation: BAG38151.1. Different initiation.
AF047182 mRNA. Translation: AAC04267.1. Different initiation.
CR456714 mRNA. Translation: CAG32995.1.
CR541997 mRNA. Translation: CAG46794.1.
BC002772 mRNA. Translation: AAH02772.1.
CCDSiCCDS33656.1.
PIRiJC5821.
RefSeqiNP_002481.2. NM_002490.3.
UniGeneiHs.274416.

3D structure databases

ProteinModelPortaliP56556.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110780. 4 interactions.
IntActiP56556. 5 interactions.
MINTiMINT-1381261.

Chemistry

ChEMBLiCHEMBL2363065.

PTM databases

PhosphoSiteiP56556.

Polymorphism and mutation databases

BioMutaiNDUFA6.
DMDMi298286909.

Proteomic databases

MaxQBiP56556.
PaxDbiP56556.
PRIDEiP56556.

Protocols and materials databases

DNASUi4700.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000498737; ENSP00000418842; ENSG00000184983.
ENST00000605916; ENSP00000475402; ENSG00000272765.
ENST00000605927; ENSP00000475816; ENSG00000273397.
ENST00000610887; ENSP00000484723; ENSG00000277365.
ENST00000617763; ENSP00000482543; ENSG00000184983.
ENST00000627617; ENSP00000485713; ENSG00000281013.
ENST00000630201; ENSP00000487431; ENSG00000281013.
GeneIDi4700.
KEGGihsa:4700.
UCSCiuc003bcb.3. human.

Organism-specific databases

CTDi4700.
GeneCardsiGC22M042481.
HGNCiHGNC:7690. NDUFA6.
HPAiHPA056934.
MIMi602138. gene.
neXtProtiNX_P56556.
PharmGKBiPA31496.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiNOG283416.
GeneTreeiENSGT00390000018898.
HOGENOMiHOG000200993.
HOVERGENiHBG002032.
InParanoidiP56556.
KOiK03950.
OMAiWKQKGHI.
PhylomeDBiP56556.
TreeFamiTF105625.

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.

Miscellaneous databases

ChiTaRSiNDUFA6. human.
GeneWikiiNDUFA6.
GenomeRNAii4700.
NextBioi18124.
PROiP56556.
SOURCEiSearch...

Gene expression databases

BgeeiP56556.
CleanExiHS_NDUFA6.
ExpressionAtlasiP56556. baseline and differential.
GenevisibleiP56556. HS.

Family and domain databases

InterProiIPR008011. Complex1_LYR.
[Graphical view]
PfamiPF05347. Complex1_LYR. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
  2. "The DNA sequence of human chromosome 22."
    Dunham I., Hunt A.R., Collins J.E., Bruskiewich R., Beare D.M., Clamp M., Smink L.J., Ainscough R., Almeida J.P., Babbage A.K., Bagguley C., Bailey J., Barlow K.F., Bates K.N., Beasley O.P., Bird C.P., Blakey S.E., Bridgeman A.M.
    , Buck D., Burgess J., Burrill W.D., Burton J., Carder C., Carter N.P., Chen Y., Clark G., Clegg S.M., Cobley V.E., Cole C.G., Collier R.E., Connor R., Conroy D., Corby N.R., Coville G.J., Cox A.V., Davis J., Dawson E., Dhami P.D., Dockree C., Dodsworth S.J., Durbin R.M., Ellington A.G., Evans K.L., Fey J.M., Fleming K., French L., Garner A.A., Gilbert J.G.R., Goward M.E., Grafham D.V., Griffiths M.N.D., Hall C., Hall R.E., Hall-Tamlyn G., Heathcott R.W., Ho S., Holmes S., Hunt S.E., Jones M.C., Kershaw J., Kimberley A.M., King A., Laird G.K., Langford C.F., Leversha M.A., Lloyd C., Lloyd D.M., Martyn I.D., Mashreghi-Mohammadi M., Matthews L.H., Mccann O.T., Mcclay J., Mclaren S., McMurray A.A., Milne S.A., Mortimore B.J., Odell C.N., Pavitt R., Pearce A.V., Pearson D., Phillimore B.J.C.T., Phillips S.H., Plumb R.W., Ramsay H., Ramsey Y., Rogers L., Ross M.T., Scott C.E., Sehra H.K., Skuce C.D., Smalley S., Smith M.L., Soderlund C., Spragon L., Steward C.A., Sulston J.E., Swann R.M., Vaudin M., Wall M., Wallis J.M., Whiteley M.N., Willey D.L., Williams L., Williams S.A., Williamson H., Wilmer T.E., Wilming L., Wright C.L., Hubbard T., Bentley D.R., Beck S., Rogers J., Shimizu N., Minoshima S., Kawasaki K., Sasaki T., Asakawa S., Kudoh J., Shintani A., Shibuya K., Yoshizaki Y., Aoki N., Mitsuyama S., Roe B.A., Chen F., Chu L., Crabtree J., Deschamps S., Do A., Do T., Dorman A., Fang F., Fu Y., Hu P., Hua A., Kenton S., Lai H., Lao H.I., Lewis J., Lewis S., Lin S.-P., Loh P., Malaj E., Nguyen T., Pan H., Phan S., Qi S., Qian Y., Ray L., Ren Q., Shaull S., Sloan D., Song L., Wang Q., Wang Y., Wang Z., White J., Willingham D., Wu H., Yao Z., Zhan M., Zhang G., Chissoe S., Murray J., Miller N., Minx P., Fulton R., Johnson D., Bemis G., Bentley D., Bradshaw H., Bourne S., Cordes M., Du Z., Fulton L., Goela D., Graves T., Hawkins J., Hinds K., Kemp K., Latreille P., Layman D., Ozersky P., Rohlfing T., Scheet P., Walker C., Wamsley A., Wohldmann P., Pepin K., Nelson J., Korf I., Bedell J.A., Hillier L.W., Mardis E., Waterston R., Wilson R., Emanuel B.S., Shaikh T., Kurahashi H., Saitta S., Budarf M.L., McDermid H.E., Johnson A., Wong A.C.C., Morrow B.E., Edelmann L., Kim U.J., Shizuya H., Simon M.I., Dumanski J.P., Peyrard M., Kedra D., Seroussi E., Fransson I., Tapia I., Bruder C.E., O'Brien K.P., Wilkinson P., Bodenteich A., Hartman K., Hu X., Khan A.S., Lane L., Tilahun Y., Wright H.
    Nature 402:489-495(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  3. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  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] OF 21-154, VARIANT VAL-35.
    Tissue: Skeletal muscle.
  5. "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] OF 26-154.
    Tissue: Heart.
  6. "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] OF 27-154.
  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] OF 27-154.
    Tissue: Lymph.
  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. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

Entry informationi

Entry nameiNDUA6_HUMAN
AccessioniPrimary (citable) accession number: P56556
Secondary accession number(s): B2RE54
, O43675, Q6FGW0, Q6IBT8, Q6IC39
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: June 15, 2010
Last modified: June 24, 2015
This is version 133 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

Caution

It is uncertain whether Met-1 or Met-27 is the initiator.Curated

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 22
    Human chromosome 22: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. 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.