InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Gene: NDUFA1

Summary

InnateDB Gene

IDBG-84068.5

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

NDUFA1

Gene Name

NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa

Synonyms

CI-MWFE; MWFE; ZNF183

Species

Homo sapiens

Ensembl Gene

ENSG00000125356

Encoded Proteins

IDBP-84070

NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa

Protein Structure

Useful resources

Stemformatics EHFPI ImmGen

Entrez Gene

Summary

The human NDUFA1 gene codes for an essential component of complex I of the respiratory chain, which transfers electrons from NADH to ubiquinone. It has been noted that the N-terminal hydrophobic domain has the potential to be folded into an alpha-helix spanning the inner mitochondrial membrane with a C-terminal hydrophilic domain interacting with globular subunits of complex I. The highly conserved two-domain structure suggests that this feature is critical for the protein function and might act as an anchor for the NADH:ubiquinone oxidoreductase complex at the inner mitochondrial membrane. However, the NDUFA1 peptide is one of about 31 components of the "hydrophobic protein" (HP) fraction of complex I which is involved in proton translocation. Thus the NDUFA1 peptide may also participate in that function. [provided by RefSeq, Jul 2008]
The human NDUFA1 gene codes for an essential component of complex I of the respiratory chain, which transfers electrons from NADH to ubiquinone. It has been noted that the N-terminal hydrophobic domain has the potential to be folded into an alpha-helix spanning the inner mitochondrial membrane with a C-terminal hydrophilic domain interacting with globular subunits of complex I. The highly conserved two-domain structure suggests that this feature is critical for the protein function and might act as an anchor for the NADH:ubiquinone oxidoreductase complex at the inner mitochondrial membrane. However, the NDUFA1 peptide is one of about 31 components of the "hydrophobic protein" (HP) fraction of complex I which is involved in proton translocation. Thus the NDUFA1 peptide may also participate in that function. [provided by RefSeq, Jul 2008]

Gene Information

Type

Protein coding

Genomic Location

Chromosome X:119871487-119876662

Strand

Forward strand

Band

q24

Transcripts

ENST00000371437

ENSP00000360492

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 8 experimentally validated interaction(s) in this database.

Experimentally validated
Total	8 [view]
Protein-Protein	5 [view]
Protein-DNA	3 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0008137	NADH dehydrogenase (ubiquinone) activity

Biological Process

GO:0006120	mitochondrial electron transport, NADH to ubiquinone
GO:0022904	respiratory electron transport chain
GO:0044237	cellular metabolic process
GO:0044281	small molecule metabolic process

Cellular Component

GO:0005739	mitochondrion
GO:0005743	mitochondrial inner membrane
GO:0005747	mitochondrial respiratory chain complex I
GO:0016021	integral component of membrane
GO:0031966	mitochondrial membrane

Orthologs

Species

Mus musculus

Bos taurus

Gene ID

Gene Order

ENSMUSG00000016427

View Gene Order

ENSBTAG00000006033

Not yet available

Pathways

NETPATH

REACTOME

REACT_22393

Respiratory electron transport pathway

REACT_6305

Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. pathway

REACT_111083

The citric acid (TCA) cycle and respiratory electron transport pathway

REACT_111217

Metabolism pathway

KEGG

hsa05010

Alzheimer's disease pathway

hsa00190

Oxidative phosphorylation pathway

hsa05012

Parkinson's disease pathway

hsa05016

Huntington's disease pathway

INOH

PID NCI

Cross-References

SwissProt

TrEMBL

UniProt Splice Variant

Entrez Gene

UniGene

RefSeq

NM_004541

HUGO

OMIM

CCDS

CCDS14590

HPRD

02093

IMGT

EMBL

GenPept

RNA Seq Atlas