U-Blot® NDUFS2 Rabbit mAb
Ucallm_WR5418-50
Price range: $268.00 through $328.00
Product details
Background:The protein encoded by this gene is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I). Mammalian mitochondrial complex I is composed of at least 43 different subunits, 7 of which are encoded by the mitochondrial genome, and the rest are the products of nuclear genes. The iron-sulfur protein fraction of complex I is made up of 7 subunits, including this gene product. Complex I catalyzes the NADH oxidation with concomitant ubiquinone reduction and proton ejection out of the mitochondria. Mutations in this gene are associated with mitochondrial complex I deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2009],
Specifications
| Size | 50μL/100μL |
| Target | NDUFS2 |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, IHC, IF, IP, ELISA |
| MW(Calculated) | 53kD |
| MW(Observed) | 49kD |
| 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 | RM1667R |
| Immunogen | The specific immunogen used to produce this antibody is proprietary information. |
| Specificity | Endogenous |
| Gene Name | NDUFS2 |
| Protein Name | NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial (Complex I-49kD) (CI-49kD) (NADH-ubiquinone oxidoreductase 49 kDa subunit) |
| Other Name | |
| Species | Human |
| Gene ID | 4720 |
| Uniprot | O75306, |
| Species.1 | Mouse |
| Gene ID.1 | |
| Uniprot.1 | Q91WD5, |
| Species.2 | Rat |
| Gene ID.2 | |
| Uniprot.2 | Q641Y2 |
| Cellular Localization | Mitochondrion inner membrane; Peripheral membrane protein; Matrix side . |
| Tissue Expression | Cerebellum,Esophagus,Muscle,Placenta, |
| 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 4Fe-4S cluster.,Disease:Defects in NDUFS2 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.,similarity:Belongs to the complex I 49 kDa subunit family.,subunit:Complex I is composed of 45 different subunits. Component of the iron-sulfur (IP) fragment of the enzyme., |
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