Recombinant Human NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 (NDUFA1), Truncated

Uniprot Id

O15239

Target Species

Human

Target Name

NDUFA1

Target Full Name

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1

Target Function

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.

Target Involvement

Mitochondrial complex I deficiency (MT-C1D)

Target Subcellular Location

Mitochondrion inner membrane; Single-pass membrane protein; Matrix side.

Target Protein Families

Complex I NDUFA1 subunit family

Target Tissue Specificity

Primarily expressed in heart and skeletal muscle.

Target Synonyms

NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1; Complex I-MWFE; CI-MWFE; NADH-ubiquinone oxidoreductase MWFE subunit

Target Background

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.