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O95299

- NDUAA_HUMAN

UniProt

O95299 - NDUAA_HUMAN

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Protein

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial

Gene

NDUFA10

Organism
Homo sapiens (Human)
Status
Reviewed - 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.

Cofactori

Binds 1 FAD per subunit.

GO - Molecular functioni

  1. ATP binding Source: InterPro
  2. NADH dehydrogenase (ubiquinone) activity Source: UniProtKB
  3. nucleoside kinase activity Source: InterPro

GO - Biological processi

  1. cellular metabolic process Source: Reactome
  2. mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
  3. respiratory electron transport chain Source: Reactome
  4. response to drug Source: Ensembl
  5. small molecule metabolic process Source: Reactome
Complete GO annotation...

Keywords - Biological processi

Electron transport, Respiratory chain, Transport

Keywords - Ligandi

FAD, Flavoprotein

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.

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
ProteomesiUP000005640: Chromosome 2

Organism-specific databases

HGNCiHGNC:7684. NDUFA10.

Subcellular locationi

GO - Cellular componenti

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

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

Orphaneti255241. Leigh syndrome with leukodystrophy.
PharmGKBiPA31490.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3535MitochondrionBy similarityAdd
BLAST
Chaini36 – 355320NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrialPRO_0000019988Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei285 – 2851N6-succinyllysineBy similarity

Proteomic databases

MaxQBiO95299.
PaxDbiO95299.
PeptideAtlasiO95299.
PRIDEiO95299.

PTM databases

PhosphoSiteiO95299.

Expressioni

Gene expression databases

BgeeiO95299.
CleanExiHS_NDUFA10.
ExpressionAtlasiO95299. baseline and differential.
GenevestigatoriO95299.

Interactioni

Subunit structurei

Complex I is composed of 45 different subunits. This a component of the hydrophobic protein fraction.1 Publication

Protein-protein interaction databases

BioGridi110785. 30 interactions.
IntActiO95299. 5 interactions.
MINTiMINT-4991752.
STRINGi9606.ENSP00000252711.

Structurei

3D structure databases

ProteinModelPortaliO95299.
SMRiO95299. Positions 60-245.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I NDUFA10 subunit family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG1428.
GeneTreeiENSGT00390000016151.
HOGENOMiHOG000267026.
HOVERGENiHBG002308.
InParanoidiO95299.
KOiK03954.
PhylomeDBiO95299.
TreeFamiTF314616.

Family and domain databases

Gene3Di3.40.50.300. 2 hits.
InterProiIPR002624. DNK.
IPR015828. NADH_UbQ_OxRdtase_42KD_su.
IPR027417. P-loop_NTPase.
[Graphical view]
PANTHERiPTHR10513. PTHR10513. 1 hit.
PfamiPF01712. dNK. 1 hit.
[Graphical view]
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. Align

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)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti2 – 21A → G.
Corresponds to variant rs11541494 [ dbSNP | Ensembl ].
VAR_034149

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei183 – 1831C → CESALQTHFWTGVAGASGKL ESGSSEEVLLINERGGRSKP G in isoform 2. 1 PublicationVSP_056417
Alternative sequencei214 – 22310Missing in isoform 2. 1 PublicationVSP_056418
Alternative sequencei334 – 35522LPGRK…WIWLK → RLDWTVCFGEESTEVKHQGH LLSVQPGTVALTVGSWLRSC LLGLHWKLLFLFPESPMHTT AFMFLC in isoform 2. 1 PublicationVSP_056419Add
BLAST

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
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.
UniGeneiHs.277677.

Genome annotation databases

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

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
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 .
CCDSi CCDS2531.1. [O95299-1 ]
PIRi JE0385.
RefSeqi NP_004535.1. NM_004544.3.
UniGenei Hs.277677.

3D structure databases

ProteinModelPortali O95299.
SMRi O95299. Positions 60-245.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 110785. 30 interactions.
IntActi O95299. 5 interactions.
MINTi MINT-4991752.
STRINGi 9606.ENSP00000252711.

Chemistry

BindingDBi O95299.
ChEMBLi CHEMBL2363065.

PTM databases

PhosphoSitei O95299.

Proteomic databases

MaxQBi O95299.
PaxDbi O95299.
PeptideAtlasi O95299.
PRIDEi O95299.

Protocols and materials databases

DNASUi 4705.
Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENST00000252711 ; ENSP00000252711 ; ENSG00000130414 . [O95299-1 ]
ENST00000307300 ; ENSP00000302321 ; ENSG00000130414 . [O95299-2 ]
GeneIDi 4705.
KEGGi hsa:4705.
UCSCi uc002vyn.3. human. [O95299-1 ]

Organism-specific databases

CTDi 4705.
GeneCardsi GC02M240854.
HGNCi HGNC:7684. NDUFA10.
MIMi 603835. gene.
neXtProti NX_O95299.
Orphaneti 255241. Leigh syndrome with leukodystrophy.
PharmGKBi PA31490.
GenAtlasi Search...

Phylogenomic databases

eggNOGi COG1428.
GeneTreei ENSGT00390000016151.
HOGENOMi HOG000267026.
HOVERGENi HBG002308.
InParanoidi O95299.
KOi K03954.
PhylomeDBi O95299.
TreeFami TF314616.

Enzyme and pathway databases

Reactomei REACT_22393. Respiratory electron transport.

Miscellaneous databases

ChiTaRSi NDUFA10. human.
GeneWikii NDUFA10.
GenomeRNAii 4705.
NextBioi 18144.
PROi O95299.
SOURCEi Search...

Gene expression databases

Bgeei O95299.
CleanExi HS_NDUFA10.
ExpressionAtlasi O95299. baseline and differential.
Genevestigatori O95299.

Family and domain databases

Gene3Di 3.40.50.300. 2 hits.
InterProi IPR002624. DNK.
IPR015828. NADH_UbQ_OxRdtase_42KD_su.
IPR027417. P-loop_NTPase.
[Graphical view ]
PANTHERi PTHR10513. PTHR10513. 1 hit.
Pfami PF01712. dNK. 1 hit.
[Graphical view ]
PIRSFi PIRSF000543. NADH_UQ_42KD. 1 hit.
SUPFAMi SSF52540. SSF52540. 1 hit.
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "cDNA of eight nuclear encoded subunits of NADH:ubiquinone oxidoreductase: human complex I cDNA characterization completed."
    Loeffen J.L.C.M., Triepels R.H., van den Heuvel L.P., Schuelke M., Buskens C.A.F., Smeets R.J.P., Trijbels J.M.F., Smeitink J.A.M.
    Biochem. Biophys. Res. Commun. 253:415-422(1998) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
  2. "Homo sapiens, NADH dehydrogenase (ubiquinone) 1 alpha subcomplex."
    Hu W., Tang L.-J., Shi Y.-W., Tian J.-Y., Jian Y.-S.
    Submitted (NOV-2001) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 2).
  3. "Generation and annotation of the DNA sequences of human chromosomes 2 and 4."
    Hillier L.W., Graves T.A., Fulton R.S., Fulton L.A., Pepin K.H., Minx P., Wagner-McPherson C., Layman D., Wylie K., Sekhon M., Becker M.C., Fewell G.A., Delehaunty K.D., Miner T.L., Nash W.E., Kremitzki C., Oddy L., Du H.
    , Sun H., Bradshaw-Cordum H., Ali J., Carter J., Cordes M., Harris A., Isak A., van Brunt A., Nguyen C., Du F., Courtney L., Kalicki J., Ozersky P., Abbott S., Armstrong J., Belter E.A., Caruso L., Cedroni M., Cotton M., Davidson T., Desai A., Elliott G., Erb T., Fronick C., Gaige T., Haakenson W., Haglund K., Holmes A., Harkins R., Kim K., Kruchowski S.S., Strong C.M., Grewal N., Goyea E., Hou S., Levy A., Martinka S., Mead K., McLellan M.D., Meyer R., Randall-Maher J., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Shah N., Swearengen-Shahid S., Snider J., Strong J.T., Thompson J., Yoakum M., Leonard S., Pearman C., Trani L., Radionenko M., Waligorski J.E., Wang C., Rock S.M., Tin-Wollam A.-M., Maupin R., Latreille P., Wendl M.C., Yang S.-P., Pohl C., Wallis J.W., Spieth J., Bieri T.A., Berkowicz N., Nelson J.O., Osborne J., Ding L., Meyer R., Sabo A., Shotland Y., Sinha P., Wohldmann P.E., Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Jones T.A., She X., Ciccarelli F.D., Izaurralde E., Taylor J., Schmutz J., Myers R.M., Cox D.R., Huang X., McPherson J.D., Mardis E.R., Clifton S.W., Warren W.C., Chinwalla A.T., Eddy S.R., Marra M.A., Ovcharenko I., Furey T.S., Miller W., Eichler E.E., Bork P., Suyama M., Torrents D., Waterston R.H., Wilson R.K.
    Nature 434:724-731(2005) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  4. "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] (ISOFORM 1).
    Tissue: Placenta.
  5. "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.
  6. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

Entry informationi

Entry nameiNDUAA_HUMAN
AccessioniPrimary (citable) accession number: O95299
Secondary accession number(s): Q8WXC9
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: May 1, 1999
Last modified: October 29, 2014
This is version 126 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 2
    Human chromosome 2: 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