功能: catalytic activity:NADH + acceptor = NAD(+) + reduced acceptor.,catalytic activity:NADH + ubiquinone = NAD(+) + ubiquinol.,cofactor:Binds 2 4Fe-4S clusters per subunit.,disease:Defects in NDUFS8 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.,function:Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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 (By similarity). May donate electrons to ubiquinone.,similarity:Belongs to the complex I 23 kDa subunit family.,similarity:Contains 2 4Fe-4S ferredoxin-type domains.,subunit:Mammalian complex I is composed of 45 different subunits.,
相关产品: RS0001,RS0002,YM3028,YM3029
细胞定位: Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side .
组织表达: Expressed in all tissues with the highest level in heart and skeletal muscle and the lowest level in lung.
功能: catalytic activity:NADH + acceptor = NAD(+) + reduced acceptor.,catalytic activity:NADH + ubiquinone = NAD(+) + ubiquinol.,cofactor:Binds 2 4Fe-4S clusters per subunit.,disease:Defects in NDUFS8 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.,function:Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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 (By similarity). May donate electrons to ubiquinone.,similarity:Belongs to the complex I 23 kDa subunit family.,similarity:Contains 2 4Fe-4S ferredoxin-type domains.,subunit:Mammalian complex I is composed of 45 different subunits.,
相关产品: RS0001,RS0002,YM3028,YM3029
细胞定位: Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side .
组织表达: Expressed in all tissues with the highest level in heart and skeletal muscle and the lowest level in lung.