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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.

GO - Molecular functioni

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

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

Electron 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

HGNCiHGNC:7692. NDUFA8.

Subcellular locationi

GO - Cellular componenti

  • mitochondrial inner membrane Source: Reactome
  • mitochondrial intermembrane space Source: UniProtKB
  • mitochondrial respiratory chain complex I Source: UniProtKB
  • mitochondrion Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA31498.

Chemistry

ChEMBLiCHEMBL2363065.

Polymorphism and mutation databases

BioMutaiNDUFA8.
DMDMi8039804.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Initiator methionineiRemovedCombined sources1 Publication
Chaini2 – 172171NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8PRO_0000118734Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Disulfide bondi36 ↔ 66Sequence analysis
Disulfide bondi46 ↔ 56Sequence analysis
Disulfide bondi78 ↔ 110Sequence analysis
Disulfide bondi88 ↔ 100Sequence analysis

Post-translational modificationi

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

Keywords - PTMi

Disulfide bond

Proteomic databases

EPDiP51970.
MaxQBiP51970.
PaxDbiP51970.
PeptideAtlasiP51970.
PRIDEiP51970.
TopDownProteomicsiP51970.

2D gel databases

SWISS-2DPAGEP51970.
UCD-2DPAGEP51970.

PTM databases

iPTMnetiP51970.
PhosphoSiteiP51970.

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.2 Publications

GO - Molecular functioni

  • protein complex binding Source: MGI

Protein-protein interaction databases

BioGridi110782. 56 interactions.
IntActiP51970. 14 interactions.
STRINGi9606.ENSP00000362873.

Structurei

3D structure databases

ProteinModelPortaliP51970.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini29 – 6941CHCH 1Add
BLAST
Domaini71 – 11343CHCH 2Add
BLAST

Motif

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Motifi36 – 4611C-X9-C motif 1Add
BLAST
Motifi56 – 6611C-X9-C motif 2Add
BLAST
Motifi78 – 8811C-X9-C motif 3Add
BLAST
Motifi100 – 11011C-X9-C motif 4Add
BLAST

Domaini

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

Sequence similaritiesi

Belongs to the complex I NDUFA8 subunit family.Curated
Contains 2 CHCH domains.Curated

Keywords - Domaini

Repeat

Phylogenomic databases

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

Family and domain databases

InterProiIPR010625. CHCH.
IPR016680. NADH_Ub_cplx-1_asu_su-8.
[Graphical view]
PfamiPF06747. CHCH. 1 hit.
[Graphical view]
PIRSFiPIRSF017016. NDUA8. 1 hit.

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)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti140 – 1401N → H in a breast cancer sample; somatic mutation. 1 Publication
VAR_036176

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF044953 mRNA. Translation: AAD42056.1.
AK314135 mRNA. Translation: BAG36825.1.
AL162423 Genomic DNA. Translation: CAH73980.1.
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

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. Translation: CAH73980.1.
CH471090 Genomic DNA. Translation: EAW87511.1.
BC001016 mRNA. Translation: AAH01016.1.
CCDSiCCDS6835.1.
RefSeqiNP_055037.1. NM_014222.2.
UniGeneiHs.495039.

3D structure databases

ProteinModelPortaliP51970.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110782. 56 interactions.
IntActiP51970. 14 interactions.
STRINGi9606.ENSP00000362873.

Chemistry

ChEMBLiCHEMBL2363065.

PTM databases

iPTMnetiP51970.
PhosphoSiteiP51970.

Polymorphism and mutation databases

BioMutaiNDUFA8.
DMDMi8039804.

2D gel databases

SWISS-2DPAGEP51970.
UCD-2DPAGEP51970.

Proteomic databases

EPDiP51970.
MaxQBiP51970.
PaxDbiP51970.
PeptideAtlasiP51970.
PRIDEiP51970.
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.
GeneCardsiNDUFA8.
HGNCiHGNC:7692. NDUFA8.
HPAiHPA041510.
HPA041600.
MIMi603359. gene.
neXtProtiNX_P51970.
PharmGKBiPA31498.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3458. Eukaryota.
ENOG4111IST. LUCA.
GeneTreeiENSGT00390000008938.
HOGENOMiHOG000090856.
HOVERGENiHBG001244.
InParanoidiP51970.
KOiK03952.
OMAiYWTCLDY.
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.
PROiP51970.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000119421.
CleanExiHS_NDUFA8.
GenevisibleiP51970. HS.

Family and domain databases

InterProiIPR010625. CHCH.
IPR016680. NADH_Ub_cplx-1_asu_su-8.
[Graphical view]
PfamiPF06747. CHCH. 1 hit.
[Graphical view]
PIRSFiPIRSF017016. NDUA8. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiNDUA8_HUMAN
AccessioniPrimary (citable) accession number: P51970
Secondary accession number(s): B1AM93, Q9Y6N0
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: January 23, 2007
Last modified: September 7, 2016
This is version 148 of the entry and version 3 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, 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. 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.