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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

  • coenzyme binding Source: InterPro
  • NADH dehydrogenase (ubiquinone) activity Source: UniProtKB
  • NADH dehydrogenase activity Source: UniProtKB
  • protein complex binding Source: MGI

GO - Biological processi

  • mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
  • mitochondrial respiratory chain complex I assembly Source: Reactome
  • sodium ion transport Source: UniProtKB
  • ubiquinone-6 biosynthetic process Source: GO_Central
Complete GO annotation...

Keywords - Biological processi

Electron transport, Respiratory chain, Transport

Keywords - Ligandi

FAD, Flavoprotein

Enzyme and pathway databases

BioCyciZFISH:HS06589-MONOMER.
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 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
Proteomesi
  • UP000005640 Componenti: Chromosome 12

Organism-specific databases

HGNCiHGNC:7693. NDUFA9.

Subcellular locationi

GO - Cellular componenti

  • mitochondrial matrix Source: UniProtKB
  • mitochondrial membrane Source: UniProtKB
  • mitochondrial respiratory chain complex I Source: UniProtKB
  • mitochondrion Source: HPA
  • nucleoplasm Source: HPA
  • nucleus Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

DisGeNETi4704.
MalaCardsiNDUFA9.
OpenTargetsiENSG00000139180.
Orphaneti2609. Isolated NADH-CoQ reductase deficiency.
PharmGKBiPA31499.

Chemistry databases

ChEMBLiCHEMBL2363065.

Polymorphism and mutation databases

BioMutaiNDUFA9.
DMDMi2833280.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 35MitochondrionBy similarityAdd BLAST35
ChainiPRO_000001999236 – 377NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrialAdd BLAST342

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei175N6-succinyllysineBy similarity1
Modified residuei189N6-acetyllysineBy similarity1
Modified residuei370N6-acetyllysineBy similarity1

Post-translational modificationi

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

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ16795.
MaxQBiQ16795.
PaxDbiQ16795.
PeptideAtlasiQ16795.
PRIDEiQ16795.
TopDownProteomicsiQ16795.

PTM databases

iPTMnetiQ16795.
PhosphoSitePlusiQ16795.
SwissPalmiQ16795.

Expressioni

Gene expression databases

BgeeiENSG00000139180.
CleanExiHS_NDUFA9.
ExpressionAtlasiQ16795. baseline and differential.
GenevisibleiQ16795. HS.

Organism-specific databases

HPAiHPA042268.
HPA073212.

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

GO - Molecular functioni

  • protein complex binding Source: MGI

Protein-protein interaction databases

BioGridi110784. 79 interactors.
DIPiDIP-38526N.
IntActiQ16795. 55 interactors.
MINTiMINT-3033599.
STRINGi9606.ENSP00000266544.

Structurei

3D structure databases

ProteinModelPortaliQ16795.
SMRiQ16795.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I NDUFA9 subunit family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2865. Eukaryota.
COG0702. LUCA.
GeneTreeiENSGT00390000006865.
HOGENOMiHOG000168008.
HOVERGENiHBG006546.
InParanoidiQ16795.
KOiK03953.
OMAiYVFAMAH.
OrthoDBiEOG091G07EJ.
PhylomeDBiQ16795.
TreeFamiTF105961.

Family and domain databases

Gene3Di3.40.50.720. 1 hit.
InterProiIPR001509. Epimerase_deHydtase.
IPR016040. NAD(P)-bd_dom.
[Graphical view]
PfamiPF01370. Epimerase. 1 hit.
[Graphical view]
SUPFAMiSSF51735. SSF51735. 1 hit.

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 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.4. human.

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.
SMRiQ16795.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110784. 79 interactors.
DIPiDIP-38526N.
IntActiQ16795. 55 interactors.
MINTiMINT-3033599.
STRINGi9606.ENSP00000266544.

Chemistry databases

ChEMBLiCHEMBL2363065.

PTM databases

iPTMnetiQ16795.
PhosphoSitePlusiQ16795.
SwissPalmiQ16795.

Polymorphism and mutation databases

BioMutaiNDUFA9.
DMDMi2833280.

Proteomic databases

EPDiQ16795.
MaxQBiQ16795.
PaxDbiQ16795.
PeptideAtlasiQ16795.
PRIDEiQ16795.
TopDownProteomicsiQ16795.

Protocols and materials databases

DNASUi4704.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000266544; ENSP00000266544; ENSG00000139180.
GeneIDi4704.
KEGGihsa:4704.
UCSCiuc001qnc.4. human.

Organism-specific databases

CTDi4704.
DisGeNETi4704.
GeneCardsiNDUFA9.
HGNCiHGNC:7693. NDUFA9.
HPAiHPA042268.
HPA073212.
MalaCardsiNDUFA9.
MIMi603834. gene.
neXtProtiNX_Q16795.
OpenTargetsiENSG00000139180.
Orphaneti2609. Isolated NADH-CoQ reductase deficiency.
PharmGKBiPA31499.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2865. Eukaryota.
COG0702. LUCA.
GeneTreeiENSGT00390000006865.
HOGENOMiHOG000168008.
HOVERGENiHBG006546.
InParanoidiQ16795.
KOiK03953.
OMAiYVFAMAH.
OrthoDBiEOG091G07EJ.
PhylomeDBiQ16795.
TreeFamiTF105961.

Enzyme and pathway databases

BioCyciZFISH:HS06589-MONOMER.
ReactomeiR-HSA-611105. Respiratory electron transport.
R-HSA-6799198. Complex I biogenesis.

Miscellaneous databases

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

Gene expression databases

BgeeiENSG00000139180.
CleanExiHS_NDUFA9.
ExpressionAtlasiQ16795. baseline and differential.
GenevisibleiQ16795. HS.

Family and domain databases

Gene3Di3.40.50.720. 1 hit.
InterProiIPR001509. Epimerase_deHydtase.
IPR016040. NAD(P)-bd_dom.
[Graphical view]
PfamiPF01370. Epimerase. 1 hit.
[Graphical view]
SUPFAMiSSF51735. SSF51735. 1 hit.
ProtoNetiSearch...

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: November 30, 2016
This is version 149 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

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 one 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.