Anti-BCKDHB antibody (183-392) (STJ112553)

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STJ112553
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Host: Rabbit
Applications: WB/ELISA
Reactivity: Mouse/Rat
Note: STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS.
Clonality : Polyclonal
Conjugation: Unconjugated
Isotype: IgG
Formulation: PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3.
Purification: Affinity purification
Concentration: Lot specific
Dilution Range: WB:1:1000-1:5000
ELISA: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: 183-392
Specificity: Recombinant fusion protein containing a sequence corresponding to amino acids 183-392 of human BCKDHB (NP_898871.1).
Immunogen Sequence: RSPWGCVGHGALYHSQSPEA FFAHCPGIKVVIPRSPFQAK GLLLSCIEDKNPCIFFEPKI LYRAAAEEVPIEPYNIPLSQ AEVIQEGSDVTLVAWGTQVH VIREVASMAKEKLGVSCEVI DLRTIIPWDVDTICKSVIKT GRLLISHEAPLTGGFASEIS STVQEECFLNLEAPISRVCG YDTPFPHIFEPFYIPDKWKC YDALRKMINY
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 - Mitochondrial
Branched-Chain Alpha-Keto Acid Dehydrogenase E1 Component Beta Chain
Bckde1b
Bckdh E1-Beta
Database Links Reactome: R-HSA-70895
Reactome: R-HSA-9859138
Cellular Localisation Mitochondrion Matrix
Alternative Antibody Names Anti-2-Oxoisovalerate Dehydrogenase Subunit Beta - Mitochondrial antibody
Anti-Branched-Chain Alpha-Keto Acid Dehydrogenase E1 Component Beta Chain antibody
Anti-Bckde1b antibody
Anti-Bckdh E1-Beta antibody
Anti-BCKDHB antibody

Information sourced from Uniprot.org