NADH dehydrogenase (ubiquinone), alpha 1

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NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa
Symbols NDUFA1 ; CI-MWFE; MWFE; ZNF183
External IDs OMIM300078 MGI1929511 HomoloGene3337 GeneCards: NDUFA1 Gene
EC number
RNA expression pattern
PBB GE NDUFA1 202298 at tn.png
More reference expression data
Species Human Mouse
Entrez 4694 54405
Ensembl ENSG00000125356 ENSMUSG00000016427
UniProt O15239 O35683
RefSeq (mRNA) NM_004541 NM_019443
RefSeq (protein) NP_004532 NP_062316
Location (UCSC) Chr X:
119.01 – 119.01 Mb
Chr X:
37.19 – 37.19 Mb
PubMed search [1] [2]

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 is a protein that in humans is encoded by the NDUFA1 gene.[1][2]


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.[2]


  1. ^ Zhuchenko O, Wehnert M, Bailey J, Sun ZS, Lee CC (Mar 1997). "Isolation, mapping, and genomic structure of an X-linked gene for a subunit of human mitochondrial complex I". Genomics 37 (3): 281–8. doi:10.1006/geno.1996.0561. PMID 8938439. 
  2. ^ a b "Entrez Gene: NDUFA1 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa". 

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