Target：NDUFS1

Fields：Oxidative phosphorylation;Metabolic pathways;Thermogenesis;Retrograde endocannabinoid signaling;Non-alcoholic fatty liver disease;Alzheimer disease;Parkinson disease;Amyotrophic lateral sclerosis;Huntington disease;Prion disease;Pathways of neurodegeneration - multiple diseases;Chemical carcinogenesis - reactive oxygen species;Diabetic cardiomyopathy

Gene Name：NDUFS1

Protein Name：NADH-ubiquinone oxidoreductase 75 kDa subunit mitochondrial

Human Gene Id：4719

Human Swiss Prot No：P28331

Mouse Gene Id：227197

Mouse Swiss Prot No：Q91VD9

Rat Gene Id：301458

Rat Swiss Prot No：Q66HF1

Immunogen：The antiserum was produced against synthesized peptide derived from human NDUFS1. AA range:620-669

Specificity：NDUFS1 Polyclonal Antibody detects endogenous levels of NDUFS1 protein.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：NDUFS1;NADH-ubiquinone oxidoreductase 75 kDa subunit; mitochondrial;Complex I-75kD;CI-75kD

Observed Band(KD)：80kD

Background： 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],

Function：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 respirato

Subcellular Location：Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side .

Expression： Brain,Cajal-Retzius cell,Colon,Fetal brain cortex,Liver,