NDUFA1

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]

Description

NADH:ubiquinone oxidoreductase subunit A1

Tissue specificity

Low tissue specificity

Subcell location

Mitochondria, Cytosol

Predicted location

Membrane

Protein family

Disease related genes
Human disease related genes
Mapped to neXtProt
Mapped to UniProt SWISS-PROT
Metabolic proteins
Predicted membrane proteins
Protein evidence (Ezkurdia et al 2014)
Protein evidence (Kim et al 2014)

Chromosome

X (119871832 - 119876662)

Ensembl ID

ENSG00000125356  (ver. 103.38)

Orthologs

Mouse Ndufa1: ENSMUSG00000016427

UniProt

O15239

neXtProt

NX_O15239

Human splice variants
Mouse splice variants

Protein structure