产品名称
NDUS8 Rabbit Polyclonal Antibody
蛋白名称
NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial (EC 1.6.5.3) (EC 1.6.99.3) (Complex I-23kD) (CI-23kD) (NADH-ubiquinone oxidoreductase 23 kDa subunit) (TYKY subunit)
存储缓冲液
Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.
Human Swissprot No.
O00217
Human Swissprot Link
https://www.uniprot.org/uniprotkb/O00217/entry
Mouse Swissprot No.
Q8K3J1
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q8K3J1
免疫原
Synthesized peptide derived from human protein . at AA range: 90-170
特异性
NDUS8 Polyclonal Antibody detects endogenous levels of protein.
稀释度
WB 1:500-2000 ELISA 1:5000-20000
宿主
Polyclonal, Rabbit,IgG
背景介绍
This gene encodes a subunit of mitochondrial NADH:ubiquinone oxidoreductase, or Complex I, a multimeric enzyme of the respiratory chain responsible for NADH oxidation, ubiquinone reduction, and the ejection of protons from mitochondria. The encoded protein is involved in the binding of two of the six to eight iron-sulfur clusters of Complex I and, as such, is required in the electron transfer process. Mutations in this gene have been associated with Leigh syndrome. [provided by RefSeq, Mar 2010],
组织表达
Expressed in all tissues with the highest level in heart and skeletal muscle and the lowest level in lung.
细胞定位
Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side .
信号通路
Oxidative phosphorylation;Alzheimer's disease;Parkinson's disease;Huntington's disease;
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.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
