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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.
Gene Name: | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa |
Synonyms: | NDUFA1, CI-MWFE, Complex I MWFE subunit, MWFE, NADH dehydrogenase, Type I dehydrogenase, ZNF183, Complex I-MWFE |
Target Sequences: | NM_004541 NP_004532.1 O15239 |
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