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Protein

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6

Gene

NDUFA6

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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.1 Publication

GO - Molecular functioni

  • NADH dehydrogenase (ubiquinone) activity Source: ProtInc

GO - Biological processi

  • mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
  • mitochondrial respiratory chain complex I assembly Source: Reactome
  • response to oxidative stress Source: UniProtKB

Keywordsi

Biological processElectron 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 alpha subcomplex subunit 6Curated
Alternative name(s):
Complex I-B14
Short name:
CI-B14
LYR motif-containing protein 6
NADH-ubiquinone oxidoreductase B14 subunit
Gene namesi
Name:NDUFA6Imported
Synonyms:LYRM6, NADHB14
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 22

Organism-specific databases

EuPathDBiHostDB:ENSG00000184983.9
HGNCiHGNC:7690 NDUFA6
MIMi602138 gene
neXtProtiNX_P56556

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi4700
OpenTargetsiENSG00000184983
PharmGKBiPA31496

Chemistry databases

ChEMBLiCHEMBL2363065
DrugBankiDB00157 NADH

Polymorphism and mutation databases

BioMutaiNDUFA6
DMDMi298286909

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001743021 – 128NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6Add BLAST128

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei11PhosphoserineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiP56556
MaxQBiP56556
PaxDbiP56556
PeptideAtlasiP56556
PRIDEiP56556
ProteomicsDBi56926
TopDownProteomicsiP56556

PTM databases

iPTMnetiP56556
PhosphoSitePlusiP56556

Expressioni

Gene expression databases

BgeeiENSG00000184983
CleanExiHS_NDUFA6
ExpressionAtlasiP56556 baseline and differential
GenevisibleiP56556 HS

Interactioni

Subunit structurei

Mammalian complex I is composed of 45 different subunits.2 Publications

Protein-protein interaction databases

BioGridi110780, 22 interactors
ComplexPortaliCPX-577 Mitochondrial respiratory chain complex I
CORUMiP56556
IntActiP56556, 57 interactors
MINTiP56556
STRINGi9606.ENSP00000418842

Structurei

Secondary structure

1128
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi25 – 40Combined sources16
Helixi43 – 49Combined sources7
Helixi56 – 66Combined sources11
Turni67 – 72Combined sources6
Helixi76 – 94Combined sources19
Helixi100 – 104Combined sources5
Turni105 – 107Combined sources3
Helixi118 – 124Combined sources7

3D structure databases

ProteinModelPortaliP56556
SMRiP56556
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I LYR family.Curated

Phylogenomic databases

eggNOGiKOG3426 Eukaryota
ENOG4111S2F LUCA
GeneTreeiENSGT00390000018898
HOGENOMiHOG000200993
HOVERGENiHBG002032
InParanoidiP56556
KOiK03950
OMAiEWIRAAP
OrthoDBiEOG091G0UGT
PhylomeDBiP56556
TreeFamiTF105625

Family and domain databases

InterProiView protein in InterPro
IPR008011 Complex1_LYR
IPR016488 NADH_Ub_cplx-1_asu_su-6
PANTHERiPTHR12964 PTHR12964, 1 hit
PfamiView protein in Pfam
PF05347 Complex1_LYR, 1 hit
PIRSFiPIRSF006643 NDUA6, 1 hit

Sequencei

Sequence statusi: Complete.

P56556-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAGSGVRQAT STASTFVKPI FSRDMNEAKR RVRELYRAWY REVPNTVHQF
60 70 80 90 100
QLDITVKMGR DKVREMFMKN AHVTDPRVVD LLVIKGKIEL EETIKVWKQR
110 120
THVMRFFHET EAPRPKDFLS KFYVGHDP
Length:128
Mass (Da):15,137
Last modified:June 20, 2018 - v4
Checksum:i5EE10C571A659D8B
GO

Sequence cautioni

The sequence CAG30415 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti6V → F in CAG32995 (Ref. 4) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0144839A → V1 PublicationCorresponds to variant dbSNP:rs1801311Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF047182 mRNA Translation: AAC04267.1
CR456529 mRNA Translation: CAG30415.1 Different initiation.
AK291874 mRNA Translation: BAF84563.1
AK316562 mRNA Translation: BAG38151.1
CR456714 mRNA Translation: CAG32995.1
CR541997 mRNA Translation: CAG46794.1
AL021878 Genomic DNA No translation available.
CH471095 Genomic DNA Translation: EAW60489.1
BC002772 mRNA Translation: AAH02772.1
PIRiJC5821
RefSeqiNP_002481.2, NM_002490.4
UniGeneiHs.274416

Genome annotation databases

EnsembliENST00000602404; ENSP00000473461; ENSG00000184983
ENST00000605916; ENSP00000475402; ENSG00000272765
ENST00000625834; ENSP00000486643; ENSG00000281013
ENST00000628740; ENSP00000486781; ENSG00000277365
ENST00000630971; ENSP00000487462; ENSG00000273397
GeneIDi4700
KEGGihsa:4700
UCSCiuc003bcb.4 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiNDUA6_HUMAN
AccessioniPrimary (citable) accession number: P56556
Secondary accession number(s): B2RE54
, O43675, Q6FGW0, Q6IBT8, Q6IC39
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: June 20, 2018
Last modified: July 18, 2018
This is version 163 of the entry and version 4 of the sequence. See 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

3D-structure, 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families

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