NADH dehydrogenase (ubiquinone), alpha 1

NDUFA1
Identifiers
Aliases	NDUFA1, CI-MWFE, MWFE, ZNF183, NADH dehydrogenase (ubiquinone), alpha 1, NADH:ubiquinone oxidoreductase subunit A1, MC1DN12
External IDs	OMIM: 300078; MGI: 1929511; HomoloGene: 3337; GeneCards: NDUFA1; OMA:NDUFA1 - orthologs
Gene location (Human)
Chr.	X chromosome (human)
End	119,876,662 bp
Gene location (Mouse)
Chr.	X chromosome (mouse)
End	36,454,816 bp
RNA expression pattern
	Top expressed in
	myocardium of left ventricle; ; right ventricle; ; apex of heart; ; right auricle; ; cardiac muscle tissue of right atrium; ; mucosa of transverse colon; ; Brodmann area 10; ; renal medulla; ; Brodmann area 9; ; muscle of thigh;
	Top expressed in
	Epithelium of choroid plexus; ; seminal vesicula; ; facial motor nucleus; ; vestibular membrane of cochlear duct; ; vestibular sensory epithelium; ; epithelium of lens; ; molar; ; right kidney; ; lobe of prostate; ; extraocular muscle;
	More reference expression data
	More reference expression data
Gene ontology
Molecular function	NADH dehydrogenase (ubiquinone) activity;
Cellular component	integral component of membrane; mitochondrion; mitochondrial respiratory chain complex I; respirasome; mitochondrial inner membrane; membrane; mitochondrial membranes; cytosol;
Biological process	mitochondrial electron transport, NADH to ubiquinone; mitochondrial respiratory chain complex I assembly;
	Sources:Amigo / QuickGO
Orthologs
	4694
	54405
	ENSG00000125356
	ENSMUSG00000016427
	O15239
	O35683
	NM_004541
	NM_019443
	NP_004532
	NP_062316
	Wikidata
View/Edit Human	View/Edit Mouse

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 is a protein that in humans is encoded by the NDUFA1 gene.^[5]^[6] The NDUFA1 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain.^[7] Mutations in the NDUFA1 gene are associated with mitochondrial Complex I deficiency.^[6]

^ ^a ^b ^c GRCh38: Ensembl release 89: ENSG00000125356 – Ensembl, May 2017
^ ^a ^b ^c GRCm38: Ensembl release 89: ENSMUSG00000016427 – Ensembl, May 2017
^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ Zhuchenko O, Wehnert M, Bailey J, Sun ZS, Lee CC (November 1996). "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.
^ ^a ^b "Entrez Gene: NDUFA1 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa".
^ Donald Voet; Judith G. Voet; Charlotte W. Pratt (2013). "18". Fundamentals of biochemistry : life at the molecular level (4th ed.). Hoboken, NJ: Wiley. pp. 581–620. ISBN 9780470547847.