Product Name:
BCKDHA-pS337
Product Number:
ab-pn810
Target Full Name: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Target Alias: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKDE1A; EC 1.2.4.4; ODBA
Product Type Specific: BCKDHA phosphosite-specific antibody
Antibody Code: PN810
Antibody Target Type: Phosphosite-specific
Antibody Phosphosite: S337
Protein UniProt: P12694
Protein SigNET: BCKDHA
Antibody Type: Polyclonal
Antibody Host Species: Rabbit
Antibody Immunogen Source: Synthetic phosphopeptide patterned after human BCKDHA
Antibody Immunogen Sequence: CGHH(pS)TSDD
Antibody Immunogen Description: Corresponds to amino acid residues G334 to D341. The effect of S337 phosphorylation is unclear. This is the second major in vivo phosphorylation sites in BCKDHA (≥83 reports from high throughput mass spectrometry studies recorded in PhosphoSitePlus). BCKDHA is known to be phosphorylated at this site in vitro by BCKDK.
Production Method: The immunizing peptide was produced by solid phase synthesis on a multipep peptide synthesizer and purified by reverse-phase hplc chromatography. Purity was assessed by analytical hplc and the amino acid sequence confirmed by mass spectrometry analysis. This peptide was coupled to KLH prior to immunization into rabbits. New Zealand White rabbits were subcutaneously injected with KLH-coupled immunizing peptide every 4 weeks for 4 months. The sera from these animals was applied onto an agarose column to which the immunogen peptide was thio-linked. Antibody was eluted from the column with 0.1 M glycine, pH 2.5. Subsequently, the antibody solution was neutralized to pH 7.0 with saturated Tris.
Antibody Modification: Unconjugated. Contact KInexus if you are interest in having the antibody biotinylated or coupled with fluorescent dyes.
Antibody Concentration: 0.9 mg/ml
Storage Buffer: Phosphate buffered saline pH 7.4, 0.05% Thimerasol
Storage Conditions: For long term storage, keep frozen at -40°C or lower. Stock solution can be kept at +4°C for more than 3 months. Avoid repeated freeze-thaw cycles.
Product Use: Western blotting | Antibody microarray
Antibody Dilution Recommended: 2 µg/ml for immunoblotting
Antibody Species Reactivity: This antibody detects the target phosphoprotein in the following species due to conservation of amino acid sequence: Human | Chimpanzee | Rhesus macaque | Dog | Rat | Mouse | Platypus | Zebra fish | Fruit fly.
Scientific Background: BCKDHA (2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial) is a 48 kDa mitochondrial enzyme that belongs to the BCKDHA family. Together with BCKDHB, it 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. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). 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. The E1 subunit catalyzes the reaction: N6-[(R)-lipoyl]-L-lysyl-[protein] + 3-methyl-2-oxobutanoate + H+ => N6-[(R)-S8-2-methylpropanoyldihydrolipoyl]-L-lysyl-[protein] + CO2. It is located in the inner mitochondrial membrane. BCKDHA is inhibited by phosphorylation at Ser-337 by BCKDK and activated by dephosphorylation by protein phosphatase PPM1K. Mutations in the BCKDHA gene hinder the catabolism of leucine, isoleucine, and valine, resulting in the accumulation of these amino acids and their toxic byproducts. This leads to Maple Syrup Urine Disease (MSUD) type 1A, a disorder characterized by severe neurotoxicity, poor feeding, and a distinct sweet odor in the urine. This description may include information annotated by UniProt and/or Google AI.