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] 1 alpha subcomplex subunit 10, mitochondrial

Gene

NDUFA10

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 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.1 Publication

Cofactori

FADNote: Binds 1 FAD per subunit.

GO - Molecular functioni

  • NADH dehydrogenase (ubiquinone) activity Source: UniProtKB

GO - Biological processi

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

Keywordsi

Biological processElectron transport, Respiratory chain, Transport
LigandFAD, Flavoprotein

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 10, mitochondrial
Alternative name(s):
Complex I-42kD
Short name:
CI-42kD
NADH-ubiquinone oxidoreductase 42 kDa subunit
Gene namesi
Name:NDUFA10
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 2

Organism-specific databases

EuPathDBiHostDB:ENSG00000130414.11
HGNCiHGNC:7684 NDUFA10
MIMi603835 gene
neXtProtiNX_O95299

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

Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Leigh syndrome (LS)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia.
See also OMIM:256000
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_078937142Q → R in LS. 1 PublicationCorresponds to variant dbSNP:rs387906873EnsemblClinVar.1

Keywords - Diseasei

Disease mutation, Leigh syndrome, Primary mitochondrial disease

Organism-specific databases

DisGeNETi4705
MalaCardsiNDUFA10
MIMi256000 phenotype
OpenTargetsiENSG00000130414
Orphaneti255241 Leigh syndrome with leukodystrophy
PharmGKBiPA31490

Chemistry databases

ChEMBLiCHEMBL2363065
DrugBankiDB00157 NADH

Polymorphism and mutation databases

BioMutaiNDUFA10

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 35MitochondrionBy similarityAdd BLAST35
ChainiPRO_000001998836 – 355NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrialAdd BLAST320

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei250Phosphoserine; by PINK1By similarity1
Modified residuei285N6-succinyllysineBy similarity1

Post-translational modificationi

Phosphorylation at Ser-250 by PINK1 is required for the binding and/or reduction of the complex I substrate ubiquinone.By similarity

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiO95299
MaxQBiO95299
PaxDbiO95299
PeptideAtlasiO95299
PRIDEiO95299
ProteomicsDBi50799

PTM databases

iPTMnetiO95299
PhosphoSitePlusiO95299
SwissPalmiO95299

Expressioni

Gene expression databases

BgeeiENSG00000130414
CleanExiHS_NDUFA10
ExpressionAtlasiO95299 baseline and differential
GenevisibleiO95299 HS

Organism-specific databases

HPAiCAB070846
HPA059529
HPA067045

Interactioni

Subunit structurei

Complex I is composed of 45 different subunits. This a component of the hydrophobic protein fraction.2 Publications

Protein-protein interaction databases

BioGridi110785, 55 interactors
ComplexPortaliCPX-577 Mitochondrial respiratory chain complex I
CORUMiO95299
IntActiO95299, 19 interactors
MINTiO95299
STRINGi9606.ENSP00000252711

Structurei

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5XTCelectron microscopy3.70w36-355[»]
5XTDelectron microscopy3.70w36-355[»]
5XTHelectron microscopy3.90w36-355[»]
5XTIelectron microscopy17.40Bw/w36-355[»]
ProteinModelPortaliO95299
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I NDUFA10 subunit family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG3877 Eukaryota
ENOG41101CN LUCA
GeneTreeiENSGT00390000016151
HOGENOMiHOG000267026
HOVERGENiHBG002308
InParanoidiO95299
KOiK03954
OMAiKPHLVIY
OrthoDBiEOG091G0WZ0
PhylomeDBiO95299
TreeFamiTF314616

Family and domain databases

CDDicd02030 NDUO42, 1 hit
InterProiView protein in InterPro
IPR031314 DNK_dom
IPR015828 NDUFA10
IPR027417 P-loop_NTPase
PfamiView protein in Pfam
PF01712 dNK, 1 hit
PIRSFiPIRSF000543 NADH_UQ_42KD, 1 hit
SUPFAMiSSF52540 SSF52540, 1 hit

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: O95299-1) [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
MALRLLKLAA TSASARVVAA GAQRVRGIHS SVQCKLRYGM WHFLLGDKAS
60 70 80 90 100
KRLTERSRVI TVDGNICTGK GKLAKEIAEK LGFKHFPEAG IHYPDSTTGD
110 120 130 140 150
GKPLATDYNG NCSLEKFYDD PRSNDGNSYR LQSWLYSSRL LQYSDALEHL
160 170 180 190 200
LTTGQGVVLE RSIFSDFVFL EAMYNQGFIR KQCVDHYNEV KSVTICDYLP
210 220 230 240 250
PHLVIYIDVP VPEVQRRIQK KGDPHEMKIT SAYLQDIENA YKKTFLPEMS
260 270 280 290 300
EKCEVLQYSA REAQDSKKVV EDIEYLKFDK GPWLKQDNRT LYHLRLLVQD
310 320 330 340 350
KFEVLNYTSI PIFLPEVTIG AHQTDRVLHQ FRELPGRKYS PGYNTEVGDK

WIWLK
Length:355
Mass (Da):40,751
Last modified:May 1, 1999 - v1
Checksum:iB5C27BC150A3E691
GO
Isoform 2 (identifier: O95299-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     183-183: C → CESALQTHFWTGVAGASGKLESGSSEEVLLINERGGRSKPG
     214-223: Missing.
     334-355: LPGRKYSPGYNTEVGDKWIWLK → RLDWTVCFGE...MHTTAFMFLC

Show »
Length:429
Mass (Da):48,563
Checksum:i37689EA2FA488544
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0341492A → G. Corresponds to variant dbSNP:rs11541494EnsemblClinVar.1
Natural variantiVAR_078937142Q → R in LS. 1 PublicationCorresponds to variant dbSNP:rs387906873EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_056417183C → CESALQTHFWTGVAGASGKL ESGSSEEVLLINERGGRSKP G in isoform 2. 1 Publication1
Alternative sequenceiVSP_056418214 – 223Missing in isoform 2. 1 Publication10
Alternative sequenceiVSP_056419334 – 355LPGRK…WIWLK → RLDWTVCFGEESTEVKHQGH LLSVQPGTVALTVGSWLRSC LLGLHWKLLFLFPESPMHTT AFMFLC in isoform 2. 1 PublicationAdd BLAST22

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF087661 mRNA Translation: AAD09755.1
AF453834 mRNA Translation: AAL50984.1
AC013469 Genomic DNA No translation available.
AC114750 Genomic DNA No translation available.
AC233275 Genomic DNA No translation available.
BC003417 mRNA Translation: AAH03417.1
CCDSiCCDS2531.1 [O95299-1]
PIRiJE0385
RefSeqiNP_004535.1, NM_004544.3 [O95299-1]
UniGeneiHs.277677

Genome annotation databases

EnsembliENST00000252711; ENSP00000252711; ENSG00000130414 [O95299-1]
ENST00000307300; ENSP00000302321; ENSG00000130414 [O95299-2]
ENST00000626224; ENSP00000487517; ENSG00000281434 [O95299-2]
ENST00000630647; ENSP00000486639; ENSG00000281434 [O95299-1]
GeneIDi4705
KEGGihsa:4705
UCSCiuc002vyn.3 human [O95299-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiNDUAA_HUMAN
AccessioniPrimary (citable) accession number: O95299
Secondary accession number(s): Q8WXC9
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: May 1, 1999
Last modified: June 20, 2018
This is version 157 of the entry and version 1 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

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health