Product Name: BCKD-2
Product Number: AB-NK257-2
Size: | 25 µg | | Price: | 89.00 |
| | | $US | |
Target Full Name: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
Target Alias: BCKD; BCKDHKIN; BCKD-kinase; Branched-chain alpha-ketoacid dehydrogenase kinase; EC 2.7.1.115; EC 2.7.11.4
Product Type Specific: Protein kinase pan-specific antibody
Antibody Code: NK257-2
Antibody Target Type: Pan-specific
Protein UniProt: O14874 Protein SigNET: O14874 Antibody Type: Polyclonal
Antibody Host Species: Rabbit
Antibody Immunogen Source: Human BCKD (BCKDK) sequence peptide Cat. No.: PE-01AZU90
Antibody Immunogen Sequence: CILHVHELYIRAFQKL
Antibody Immunogen Description: Corresponds to amino acid residues I122 to L136
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: 1 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: Human; Mouse
Scientific Background: BCKD is a protein-serine/threonine kinase of the Atypical group and PDHK family. Its is associated with the mitochondrial matrix and features a HATPase_c domain. BCKD dimerizes through direct interaction of two opposing nucleotide-binding domains. It phosphorylates and inactivates the E1-alpha subunit of the branched-chain ketoacid dehydrogenase (BCKDK), which is involved in the catabolism of valine, leucine and isoleucine. Inactivating mutations in BCKD have been discovered in families with autism, epilepsy, and intellectual disability, a syndrome that may respond to dietary supplementation.