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Protein

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial

Gene

NDUFA9

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.

Cofactori

FADNote: Binds 1 FAD per subunit.

GO - Molecular functioni

  1. coenzyme binding Source: InterPro
  2. NADH dehydrogenase (ubiquinone) activity Source: UniProtKB
  3. NADH dehydrogenase activity Source: UniProtKB
  4. protein complex binding Source: MGI

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. small molecule metabolic process Source: Reactome
  5. sodium ion transport Source: UniProtKB
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 9, mitochondrial
Alternative name(s):
Complex I-39kD
Short name:
CI-39kD
NADH-ubiquinone oxidoreductase 39 kDa subunit
Gene namesi
Name:NDUFA9
Synonyms:NDUFS2L
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componenti: Chromosome 12

Organism-specific databases

HGNCiHGNC:7693. NDUFA9.

Subcellular locationi

GO - Cellular componenti

  1. mitochondrial inner membrane Source: Reactome
  2. mitochondrial matrix Source: UniProtKB
  3. mitochondrial membrane Source: UniProtKB
  4. mitochondrial respiratory chain complex I Source: UniProtKB
  5. nucleus Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

Orphaneti2609. Isolated NADH-CoQ reductase deficiency.
PharmGKBiPA31499.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3535MitochondrionBy similarityAdd
BLAST
Chaini36 – 377342NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrialPRO_0000019992Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei175 – 1751N6-succinyllysineBy similarity
Modified residuei189 – 1891N6-acetyllysineBy similarity
Modified residuei370 – 3701N6-acetyllysineBy similarity

Post-translational modificationi

Acetylated on lysine residues. BLOC1S1 is required for acetylation.1 Publication

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiQ16795.
PaxDbiQ16795.
PRIDEiQ16795.

PTM databases

PhosphoSiteiQ16795.

Expressioni

Gene expression databases

BgeeiQ16795.
CleanExiHS_NDUFA9.
ExpressionAtlasiQ16795. baseline and differential.
GenevestigatoriQ16795.

Organism-specific databases

HPAiHPA042268.

Interactioni

Subunit structurei

Complex I is composed of 45 different subunits. This a component of the hydrophobic protein fraction. Interacts with BLOC1S1. Interacts with SLC2A4 (By similarity).By similarity

Binary interactionsi

WithEntry#Exp.IntActNotes
BLOC1S1P785373EBI-1045087,EBI-348630
NOA1Q8NC602EBI-1045087,EBI-717871

Protein-protein interaction databases

BioGridi110784. 24 interactions.
DIPiDIP-38526N.
IntActiQ16795. 15 interactions.
MINTiMINT-3033599.
STRINGi9606.ENSP00000266544.

Structurei

3D structure databases

ProteinModelPortaliQ16795.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I NDUFA9 subunit family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG0702.
GeneTreeiENSGT00390000006865.
HOGENOMiHOG000168008.
HOVERGENiHBG006546.
InParanoidiQ16795.
KOiK03953.
OMAiGREWETK.
PhylomeDBiQ16795.
TreeFamiTF105961.

Family and domain databases

Gene3Di3.40.50.720. 1 hit.
InterProiIPR001509. Epimerase_deHydtase_N.
IPR016040. NAD(P)-bd_dom.
[Graphical view]
PfamiPF01370. Epimerase. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

Q16795-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAAAAQSRVV RVLSMSRSAI TAIATSVCHG PPCRQLHHAL MPHGKGGRSS
60 70 80 90 100
VSGIVATVFG ATGFLGRYVV NHLGRMGSQV IIPYRCDKYD IMHLRPMGDL
110 120 130 140 150
GQLLFLEWDA RDKDSIRRVV QHSNVVINLI GRDWETKNFD FEDVFVKIPQ
160 170 180 190 200
AIAQLSKEAG VEKFIHVSHL NANIKSSSRY LRNKAVGEKV VRDAFPEAII
210 220 230 240 250
VKPSDIFGRE DRFLNSFASM HRFGPIPLGS LGWKTVKQPV YVVDVSKGIV
260 270 280 290 300
NAVKDPDANG KSFAFVGPSR YLLFHLVKYI FAVAHRLFLP FPLPLFAYRW
310 320 330 340 350
VARVFEISPF EPWITRDKVE RMHITDMKLP HLPGLEDLGI QATPLELKAI
360 370
EVLRRHRTYR WLSAEIEDVK PAKTVNI
Length:377
Mass (Da):42,510
Last modified:November 1, 1997 - v2
Checksum:i66A1CC7FCE86DD0E
GO

Sequence cautioni

The sequence CAA54099.1 differs from that shown. Reason: Frameshift at position 18. Curated

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF050641 mRNA. Translation: AAD42055.1.
BC009311 mRNA. Translation: AAH09311.1.
BC015837 mRNA. Translation: AAH15837.1.
BC111546 mRNA. Translation: AAI11547.1.
X76665 Genomic DNA. Translation: CAA54099.1. Frameshift.
L04490 mRNA. Translation: AAA36350.1.
CCDSiCCDS8532.1.
PIRiI37258.
RefSeqiNP_004993.1. NM_005002.4.
UniGeneiHs.75227.

Genome annotation databases

EnsembliENST00000266544; ENSP00000266544; ENSG00000139180.
GeneIDi4704.
KEGGihsa:4704.
UCSCiuc001qnc.3. human.

Polymorphism databases

DMDMi2833280.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF050641 mRNA. Translation: AAD42055.1.
BC009311 mRNA. Translation: AAH09311.1.
BC015837 mRNA. Translation: AAH15837.1.
BC111546 mRNA. Translation: AAI11547.1.
X76665 Genomic DNA. Translation: CAA54099.1. Frameshift.
L04490 mRNA. Translation: AAA36350.1.
CCDSiCCDS8532.1.
PIRiI37258.
RefSeqiNP_004993.1. NM_005002.4.
UniGeneiHs.75227.

3D structure databases

ProteinModelPortaliQ16795.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110784. 24 interactions.
DIPiDIP-38526N.
IntActiQ16795. 15 interactions.
MINTiMINT-3033599.
STRINGi9606.ENSP00000266544.

Chemistry

ChEMBLiCHEMBL2363065.

PTM databases

PhosphoSiteiQ16795.

Polymorphism databases

DMDMi2833280.

Proteomic databases

MaxQBiQ16795.
PaxDbiQ16795.
PRIDEiQ16795.

Protocols and materials databases

DNASUi4704.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000266544; ENSP00000266544; ENSG00000139180.
GeneIDi4704.
KEGGihsa:4704.
UCSCiuc001qnc.3. human.

Organism-specific databases

CTDi4704.
GeneCardsiGC12P004760.
HGNCiHGNC:7693. NDUFA9.
HPAiHPA042268.
MIMi603834. gene.
neXtProtiNX_Q16795.
Orphaneti2609. Isolated NADH-CoQ reductase deficiency.
PharmGKBiPA31499.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiCOG0702.
GeneTreeiENSGT00390000006865.
HOGENOMiHOG000168008.
HOVERGENiHBG006546.
InParanoidiQ16795.
KOiK03953.
OMAiGREWETK.
PhylomeDBiQ16795.
TreeFamiTF105961.

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.

Miscellaneous databases

ChiTaRSiNDUFA9. human.
GeneWikiiNDUFA9.
GenomeRNAii4704.
NextBioi18140.
PROiQ16795.
SOURCEiSearch...

Gene expression databases

BgeeiQ16795.
CleanExiHS_NDUFA9.
ExpressionAtlasiQ16795. baseline and differential.
GenevestigatoriQ16795.

Family and domain databases

Gene3Di3.40.50.720. 1 hit.
InterProiIPR001509. Epimerase_deHydtase_N.
IPR016040. NAD(P)-bd_dom.
[Graphical view]
PfamiPF01370. Epimerase. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "39 kDa subunit of NADH-ubiquinone oxidoreductase."
    Loeffen J.L.C.M., Smeets R.J.P., Triepels R., Ruitenbeek W., Smeitink J.A.M., van den Heuvel L.
    Submitted (JAN-1998) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
  2. "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(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Colon, Muscle and Skeletal muscle.
  3. "Purification of CpG islands using a methylated DNA binding column."
    Cross S.H., Charlton J.A., Nan X., Bird A.P.
    Nat. Genet. 6:236-244(1993) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-33.
    Tissue: Blood.
  4. "Construction and evaluation of a hncDNA library of human 12p transcribed sequences derived from a somatic cell hybrid."
    Baens M., Chaffanet M., Cassiman J.-J., van den Berghe H., Marynen P.
    Genomics 16:214-218(1992) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 3-377.
    Tissue: Liver.
  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(2002) [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].
  7. "Identification of a molecular component of the mitochondrial acetyl transferase program; a novel role for GCN5L1."
    Scott I., Webster B.R., Li J.H., Sack M.N.
    Biochem. J. 443:655-661(2011) [PubMed] [Europe PMC] [Abstract]
    Cited for: INTERACTION WITH BLOC1S1, ACETYLATION.
  8. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
    Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.
    J. Proteomics 96:253-262(2013) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

Entry informationi

Entry nameiNDUA9_HUMAN
AccessioniPrimary (citable) accession number: Q16795
Secondary accession number(s): Q14076, Q2NKX0
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: November 1, 1997
Last modified: March 4, 2015
This is version 130 of the entry and version 2 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 12
    Human chromosome 12: 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

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.