Pyruvate Dehydrogenase E1 beta subunit Recombinant Rabbit Monoclonal Antibody [JM66-33]
CAT:
992-ET1705-57
Size:
100 µL
Price:
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- Availability: 24/48H Stock Items & 2 to 6 Weeks non Stock Items.
- Dry Ice Shipment: No
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Pyruvate Dehydrogenase E1 beta subunit Recombinant Rabbit Monoclonal Antibody [JM66-33]
- Description: The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO. It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). Defects in PDHB are a cause of pyruvate dehydrogenase E1 component deficiency (PDHE1 deficiency). PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (LS) (Leigh encephalomyelopathy).
- Product Name Alternative: DKFZp564K0164 antibody mitochondrial antibody ODPB_HUMAN antibody pdhB antibody PDHBD antibody PDHE1 B antibody PDHE1-B antibody PHE1B antibody Pyruvate dehydrogenase (lipoamide) beta antibody Pyruvate dehydrogenase E1 beta polypeptide antibody Pyruvate dehydrogenase E1 component subunit beta antibody Pyruvate dehydrogenase E1 component subunit beta mitochondrial antibody
- Abbreviation: PDHE1-B, PDHB, PHE1B
- CAS Number: 9000-83-3
- Swiss Prot: P11177 Human, Q9D051 Mouse, P49432 Rat
- Cellular Locus: Mitochondrion matrix.
- Host: Rabbit
- Species Reactivity: Human, Mouse, Rat
- Immunogen: Synthetic peptide within Human Pyruvate Dehydrogenase E1 beta subunit aa 310-359 / 359.
- Isotype: IgG
- Conjugation: Non-conjugated
- Type: Recombinant Rabbit monoclonal Antibody
- Applications: WB, IHC-P, FC, IP
- Positive Control: HepG2, Hela, PC-12, mouse kidney, rat heart tissue, human kidney tissue, human stomach cancer tissue, mouse hippocampus tissue, mouse brain tissue.
- Concentration: 1 mg/mL
- Dilution: WB: 1:500-1:2,000 ;IHC-P: 1:50-1:200 ;FC: 1:50-1:100 ;IP: Use at an assay dependent concentration.
- Purity: Protein A affinity purified.
- Form: Liquid
- Buffer: 1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
- Molecular Weight: Predicted band size: 39 kDa
- Storage Conditions: Store at +4°C after thawing. Aliquot store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
- Recombinant Antibody: Yes