Name: U-Blot® NDUFS1 Rabbit mAb
SKU: WR4664
Price: 268 USD
Availability: InStock

Product details

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],

Specifications

Size	50μL/100μL
Target	NDUFS1
Reactivity	Human, Mouse, Rat
Applications	WB, IHC, IF, IP, ELISA
MW(Calculated)	80kD
MW(Observed)	77kD
Host Species	Rabbit
Isotype	IgG,Kappa
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	IHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
Formulation	PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
Purification	Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
Clonality	Monoclonal
Clone Number	RM0942R
Immunogen	The specific immunogen used to produce this antibody is proprietary information.
Specificity	Endogenous
Gene Name	NDUFS1
Protein Name	NADH-ubiquinone oxidoreductase 75 kDa subunit mitochondrial
Other Name	NDUFS1; / NADH-ubiquinone oxidoreductase 75 kDa subunit; / mitochondrial; / Complex I-75kD; / CI-75kD
Species	Human
Gene ID	4719
Uniprot	P28331,
Species.1	Mouse
Gene ID.1	227197
Uniprot.1	Q91VD9,
Species.2	Rat
Gene ID.2	301458
Uniprot.2	Q66HF1
Cellular Localization	Mitochondrion inner membrane; Peripheral membrane protein; Matrix side .
Tissue Expression	Brain,Cajal-Retzius cell,Colon,Fetal brain cortex,Liver,
Signaling_pathway	Human Diseases >> Neurodegenerative disease >> Alzheimer disease;Human Diseases >> Neurodegenerative disease >> Parkinson disease;Human Diseases >> Neurodegenerative disease >> Amyotrophic lateral sclerosis;Human Diseases >> Neurodegenerative disease >> Huntington disease;Human Diseases >> Neurodegenerative disease >> Prion disease;Human Diseases >> Neurodegenerative disease >> Pathways of neurodegeneration - multiple diseases
Research Areas	>>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
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 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.,