Host: | Rabbit |
Applications: | WB/ELISA |
Reactivity: | Human/Mouse/Rat |
Note: | STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
Clonality : | Monoclonal |
Clone ID : | S5256RM |
Conjugation: | Unconjugated |
Isotype: | IgG |
Formulation: | PBS with 0.05% Proclin300, 0.05% BSA, 50% Glycerol, pH 7.3. |
Purification: | Affinity purification |
Concentration: | Lot specific |
Dilution Range: | WB:1:2000-1:12000ELISA:Recommended starting concentration is 1 Mu g/mL. Please optimize the concentration based on your specific assay requirements. |
Storage Instruction: | Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles. |
Gene Symbol: | BCKDHB |
Gene ID: | 594 |
Uniprot ID: | ODBB_HUMAN |
Immunogen Region: | 51-392 |
Specificity: | Recombinant fusion protein containing a sequence corresponding to amino acids 51-392 of human BCKDHB (NP_898871.1). |
Immunogen Sequence: | VAHFTFQPDPEPREYGQTQK MNLFQSVTSALDNSLAKDPT AVIFGEDVAFGGVFRCTVGL RDKYGKDRVFNTPLCEQGIV GFGIGIAVTGATAIAEIQFA DYIFPAFDQIVNEAAKYRYR SGDLFNCGSLTIRSPWGCVG HGALYHSQSPEAFFAHCPGI KVVIPRSPFQAKGLLLSCIE DKNPCIFFEPKILYRAAAEE VPIEPYNIPLSQAEVIQEGS DVTLVAWGTQVHVIREVAS |
Function | Together with BCKDHA forms the heterotetrameric E1 subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex. The BCKD complex catalyzes the multi-step oxidative decarboxylation of alpha-ketoacids derived from the branched-chain amino-acids valine, leucine and isoleucine producing CO2 and acyl-CoA which is subsequently utilized to produce energy. The E1 subunit catalyzes the first step with the decarboxylation of the alpha-ketoacid forming an enzyme-product intermediate. A reductive acylation mediated by the lipoylamide cofactor of E2 extracts the acyl group from the E1 active site for the next step of the reaction. |
Protein Name | 2-Oxoisovalerate Dehydrogenase Subunit Beta - MitochondrialBranched-Chain Alpha-Keto Acid Dehydrogenase E1 Component Beta ChainBckde1bBckdh E1-Beta |
Database Links | Reactome: R-HSA-70895Reactome: R-HSA-9859138 |
Cellular Localisation | Mitochondrion Matrix |
Alternative Antibody Names | Anti-2-Oxoisovalerate Dehydrogenase Subunit Beta - Mitochondrial antibodyAnti-Branched-Chain Alpha-Keto Acid Dehydrogenase E1 Component Beta Chain antibodyAnti-Bckde1b antibodyAnti-Bckdh E1-Beta antibodyAnti-BCKDHB antibody |
Information sourced from Uniprot.org