Molecular weight (DA)
80kD
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.
Dilution rate
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/40000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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
Protein name
NADH-ubiquinone oxidoreductase 75 kDa subunit mitochondrial
Other name
NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit; mitochondrial; Complex I-75kD; CI-75kD
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
Human protein sequence Database
P28331
Mouse protein sequence database
Q91VD9
Rat protein sequence database
Q66HF1
Cellular localization
Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side .
Tissue expression
Brain,Cajal-Retzius cell,Colon,Fetal brain cortex,Liver,
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