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Protein

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8

Gene

NDUFA8

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

GO - Molecular functioni

  • NADH dehydrogenase (ubiquinone) activity Source: ProtInc
  • protein-containing complex binding Source: MGI

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

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 8
Alternative name(s):
Complex I-19kD
Short name:
CI-19kD
Complex I-PGIV
Short name:
CI-PGIV
NADH-ubiquinone oxidoreductase 19 kDa subunit
Gene namesi
Name:NDUFA8
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 9

Organism-specific databases

EuPathDBiHostDB:ENSG00000119421.6
HGNCiHGNC:7692 NDUFA8
MIMi603359 gene
neXtProtiNX_P51970

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

DisGeNETi4702
OpenTargetsiENSG00000119421
PharmGKBiPA31498

Chemistry databases

ChEMBLiCHEMBL2363065
DrugBankiDB00157 NADH

Polymorphism and mutation databases

BioMutaiNDUFA8
DMDMi8039804

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources1 Publication
ChainiPRO_00001187342 – 172NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8Add BLAST171

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi36 ↔ 66PROSITE-ProRule annotation1 Publication
Disulfide bondi46 ↔ 56PROSITE-ProRule annotation1 Publication
Disulfide bondi78 ↔ 110PROSITE-ProRule annotation1 Publication
Disulfide bondi88 ↔ 100PROSITE-ProRule annotation1 Publication

Post-translational modificationi

May contain intrachain disulfide bonds, as evidenced by its electrophoretic mobility under reducing vs non-reducing conditions.1 Publication

Keywords - PTMi

Disulfide bond

Proteomic databases

EPDiP51970
MaxQBiP51970
PaxDbiP51970
PeptideAtlasiP51970
PRIDEiP51970
ProteomicsDBi56464
TopDownProteomicsiP51970

2D gel databases

SWISS-2DPAGEiP51970
UCD-2DPAGEiP51970

PTM databases

iPTMnetiP51970
PhosphoSitePlusiP51970

Expressioni

Gene expression databases

BgeeiENSG00000119421 Expressed in 223 organ(s), highest expression level in quadriceps femoris
CleanExiHS_NDUFA8
GenevisibleiP51970 HS

Organism-specific databases

HPAiHPA041510
HPA041600

Interactioni

Subunit structurei

Complex I is composed of 45 different subunits.3 Publications

Protein-protein interaction databases

BioGridi110782, 85 interactors
ComplexPortaliCPX-577 Mitochondrial respiratory chain complex I
CORUMiP51970
IntActiP51970, 29 interactors
MINTiP51970
STRINGi9606.ENSP00000362873

Structurei

3D structure databases

ProteinModelPortaliP51970
SMRiP51970
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini33 – 74CHCH 1PROSITE-ProRule annotationAdd BLAST42
Domaini75 – 118CHCH 2PROSITE-ProRule annotationAdd BLAST44

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi36 – 46Cx9C motif 1PROSITE-ProRule annotationAdd BLAST11
Motifi56 – 66Cx9C motif 2PROSITE-ProRule annotationAdd BLAST11
Motifi78 – 88Cx9C motif 3PROSITE-ProRule annotationAdd BLAST11
Motifi100 – 110Cx9C motif 4PROSITE-ProRule annotationAdd BLAST11

Domaini

Contains four C-X9-C motifs that are predicted to form a helix-coil-helix structure, permitting the formation of intramolecular disulfide bonds.1 Publication

Sequence similaritiesi

Belongs to the complex I NDUFA8 subunit family.Curated

Keywords - Domaini

Repeat

Phylogenomic databases

eggNOGiKOG3458 Eukaryota
ENOG4111IST LUCA
GeneTreeiENSGT00390000008938
HOGENOMiHOG000090856
HOVERGENiHBG001244
InParanoidiP51970
KOiK03952
OMAiNEDFMLC
OrthoDBiEOG091G0M0S
PhylomeDBiP51970
TreeFamiTF105633

Family and domain databases

InterProiView protein in InterPro
IPR010625 CHCH
IPR016680 NDUFA8
PANTHERiPTHR13344 PTHR13344, 1 hit
PfamiView protein in Pfam
PF06747 CHCH, 1 hit
PIRSFiPIRSF017016 NDUA8, 1 hit
PROSITEiView protein in PROSITE
PS51808 CHCH, 2 hits

Sequencei

Sequence statusi: Complete.

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

P51970-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MPGIVELPTL EELKVDEVKI SSAVLKAAAH HYGAQCDKPN KEFMLCRWEE
60 70 80 90 100
KDPRRCLEEG KLVNKCALDF FRQIKRHCAE PFTEYWTCID YTGQQLFRHC
110 120 130 140 150
RKQQAKFDEC VLDKLGWVRP DLGELSKVTK VKTDRPLPEN PYHSRPRPDP
160 170
SPEIEGDLQP ATHGSRFYFW TK
Length:172
Mass (Da):20,105
Last modified:January 23, 2007 - v3
Checksum:iE1647472B69E1149
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_036176140N → H in a breast cancer sample; somatic mutation. 1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF044953 mRNA Translation: AAD42056.1
AK314135 mRNA Translation: BAG36825.1
AL162423 Genomic DNA No translation available.
CH471090 Genomic DNA Translation: EAW87511.1
BC001016 mRNA Translation: AAH01016.1
CCDSiCCDS6835.1
RefSeqiNP_055037.1, NM_014222.2
UniGeneiHs.495039

Genome annotation databases

EnsembliENST00000373768; ENSP00000362873; ENSG00000119421
GeneIDi4702
KEGGihsa:4702
UCSCiuc004blv.4 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF044953 mRNA Translation: AAD42056.1
AK314135 mRNA Translation: BAG36825.1
AL162423 Genomic DNA No translation available.
CH471090 Genomic DNA Translation: EAW87511.1
BC001016 mRNA Translation: AAH01016.1
CCDSiCCDS6835.1
RefSeqiNP_055037.1, NM_014222.2
UniGeneiHs.495039

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5XTCelectron microscopy3.70u4-172[»]
5XTDelectron microscopy3.70u4-172[»]
5XTHelectron microscopy3.90u4-172[»]
5XTIelectron microscopy17.40Bu/u4-172[»]
ProteinModelPortaliP51970
SMRiP51970
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110782, 85 interactors
ComplexPortaliCPX-577 Mitochondrial respiratory chain complex I
CORUMiP51970
IntActiP51970, 29 interactors
MINTiP51970
STRINGi9606.ENSP00000362873

Chemistry databases

ChEMBLiCHEMBL2363065
DrugBankiDB00157 NADH

PTM databases

iPTMnetiP51970
PhosphoSitePlusiP51970

Polymorphism and mutation databases

BioMutaiNDUFA8
DMDMi8039804

2D gel databases

SWISS-2DPAGEiP51970
UCD-2DPAGEiP51970

Proteomic databases

EPDiP51970
MaxQBiP51970
PaxDbiP51970
PeptideAtlasiP51970
PRIDEiP51970
ProteomicsDBi56464
TopDownProteomicsiP51970

Protocols and materials databases

DNASUi4702
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000373768; ENSP00000362873; ENSG00000119421
GeneIDi4702
KEGGihsa:4702
UCSCiuc004blv.4 human

Organism-specific databases

CTDi4702
DisGeNETi4702
EuPathDBiHostDB:ENSG00000119421.6
GeneCardsiNDUFA8
HGNCiHGNC:7692 NDUFA8
HPAiHPA041510
HPA041600
MIMi603359 gene
neXtProtiNX_P51970
OpenTargetsiENSG00000119421
PharmGKBiPA31498
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3458 Eukaryota
ENOG4111IST LUCA
GeneTreeiENSGT00390000008938
HOGENOMiHOG000090856
HOVERGENiHBG001244
InParanoidiP51970
KOiK03952
OMAiNEDFMLC
OrthoDBiEOG091G0M0S
PhylomeDBiP51970
TreeFamiTF105633

Enzyme and pathway databases

ReactomeiR-HSA-611105 Respiratory electron transport
R-HSA-6799198 Complex I biogenesis

Miscellaneous databases

ChiTaRSiNDUFA8 human
GeneWikiiNDUFA8
GenomeRNAii4702
PROiPR:P51970
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000119421 Expressed in 223 organ(s), highest expression level in quadriceps femoris
CleanExiHS_NDUFA8
GenevisibleiP51970 HS

Family and domain databases

InterProiView protein in InterPro
IPR010625 CHCH
IPR016680 NDUFA8
PANTHERiPTHR13344 PTHR13344, 1 hit
PfamiView protein in Pfam
PF06747 CHCH, 1 hit
PIRSFiPIRSF017016 NDUA8, 1 hit
PROSITEiView protein in PROSITE
PS51808 CHCH, 2 hits
ProtoNetiSearch...

Entry informationi

Entry nameiNDUA8_HUMAN
AccessioniPrimary (citable) accession number: P51970
Secondary accession number(s): B1AM93, Q9Y6N0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: January 23, 2007
Last modified: November 7, 2018
This is version 167 of the entry and version 3 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, Direct protein sequencing, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  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. Human chromosome 9
    Human chromosome 9: entries, gene names and cross-references to MIM
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Main funding by: National Institutes of Health

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