NDUFS3

NDUFS3
Identifiers
Aliases	NDUFS3, CI-30, NADH:ubiquinone oxidoreductase core subunit S3, MC1DN8
External IDs	OMIM: 603846; MGI: 1915599; HomoloGene: 3346; GeneCards: NDUFS3; OMA:NDUFS3 - orthologs
Gene location (Human)
Chr.	Chromosome 11 (human)
End	47,584,562 bp
RNA expression pattern
	Top expressed in
	putamen; ; mucosa of transverse colon; ; apex of heart; ; muscle of thigh; ; primary visual cortex; ; caudate nucleus; ; left ventricle; ; gastrocnemius muscle; ; prefrontal cortex; ; Brodmann area 9;
	n/a
	More reference expression data
	More reference expression data
Gene ontology
Molecular function	oxidoreductase activity, acting on NAD(P)H; protein binding; electron transfer activity; oxidoreductase activity; NADH dehydrogenase activity; NADH dehydrogenase (ubiquinone) activity;
Cellular component	membrane; mitochondrial membranes; myelin sheath; mitochondrial respiratory chain complex I; mitochondrial matrix; mitochondrion; mitochondrial inner membrane; respirasome; nuclear body;
Biological process	substantia nigra development; negative regulation of intrinsic apoptotic signaling pathway; reactive oxygen species metabolic process; mitochondrial respiratory chain complex I assembly; negative regulation of cell growth; mitochondrial electron transport, NADH to ubiquinone; cellular respiration;
	Sources:Amigo / QuickGO
Orthologs
	4722
	68349
	ENSG00000213619; ENSG00000285387
	ENSMUSG00000005510
	O75489
	Q9DCT2
	NM_004551
	NM_026688
	NP_004542
	NP_080964
	Wikidata
View/Edit Human	View/Edit Mouse

NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial is an enzyme that in humans is encoded by the NDUFS3 gene on chromosome 11. This gene encodes one of the iron-sulfur protein (IP) components of mitochondrial NADH:ubiquinone oxidoreductase (complex I). Mutations in this gene are associated with Leigh syndrome resulting from mitochondrial complex I deficiency.