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Protein

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8

Gene

NDUFA8

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.1 Publication

GO - Molecular functioni

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

GO - Biological processi

  • mitochondrial electron transport, NADH to ubiquinone Source: Reactome
  • 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.

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 annotation
Disulfide bondi46 ↔ 56PROSITE-ProRule annotation
Disulfide bondi78 ↔ 110PROSITE-ProRule annotation
Disulfide bondi88 ↔ 100PROSITE-ProRule annotation

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.
TopDownProteomicsiP51970.

2D gel databases

SWISS-2DPAGEiP51970.
UCD-2DPAGEiP51970.

PTM databases

iPTMnetiP51970.
PhosphoSitePlusiP51970.

Expressioni

Gene expression databases

BgeeiENSG00000119421.
CleanExiHS_NDUFA8.
GenevisibleiP51970. HS.

Organism-specific databases

HPAiHPA041510.
HPA041600.

Interactioni

Subunit structurei

Complex I is composed of 45 different subunits.3 Publications

GO - Molecular functioni

  • protein complex binding Source: MGI

Protein-protein interaction databases

BioGridi110782. 65 interactors.
CORUMiP51970.
IntActiP51970. 16 interactors.
STRINGi9606.ENSP00000362873.

Structurei

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...

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.
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

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: October 25, 2017
This is version 158 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. Human chromosome 9
    Human chromosome 9: 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families