产品名称
NDUFS1 Rabbit Polyclonal Antibody
别名
NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit; mitochondrial; Complex I-75kD; CI-75kD
蛋白名称
NADH-ubiquinone oxidoreductase 75 kDa subunit mitochondrial
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=4719
Human Swissprot No.
P28331
Human Swissprot Link
http://www.uniprot.org/uniprotkb/P28331/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=227197
Mouse Swissprot No.
Q91VD9
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q91VD9
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=301458
Rat Swissprot Link
http://www.uniprot.org/uniprot/Q66HF1
免疫原
The antiserum was produced against synthesized peptide derived from human NDUFS1. AA range:620-669
特异性
NDUFS1 Polyclonal Antibody detects endogenous levels of NDUFS1 protein.
稀释度
WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200
宿主
Polyclonal, Rabbit,IgG
背景介绍
The protein encoded by this gene belongs to the complex I 75 kDa subunit family. Mammalian complex I is composed of 45 different subunits. It locates at the mitochondrial inner membrane. This protein has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. This protein is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. Mutations in this gene are associated with complex I deficiency. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jan 2011],
组织表达
Brain,Cajal-Retzius cell,Colon,Fetal brain cortex,Liver,
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 1 2Fe-2S cluster per subunit.,cofactor:Binds 2 4Fe-4S clusters per subunit.,disease:Defects in NDUFS1 are a cause of complex I mitochondrial respiratory chain deficiency [MIM:252010]. Complex I (NADH-ubiquinone oxidoreductase), the largest complex of the mitochondrial respiratory chain, contains more than 40 subunits. It is embedded in the inner mitochondrial membrane and is partly protruding in the matrix. Complex I deficiency is the most common cause of mitochondrial disorders. It represents largely one-third of all cases of respiratory chain deficiency and is responsible for a variety of clinical symptoms, ranging from neurological disorders to cardiomyopathy, liver failure, and myopathy.,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). This is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized.,similarity:Belongs to the complex I 75 kDa subunit family.,similarity:Contains 1 2Fe-2S ferredoxin-type domain.,subunit: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.