Product Name: BCKD-1
Product Number: AB-NK257-1
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: NK257-1

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-01AZT85
Antibody Immunogen Sequence: CQSAIDAAAEKPSVRL

Antibody Immunogen Description: Corresponds to amino acid residues Q56 to L70

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

Antibody Positive Control: The observed molecular mass of the processed target protein on SDS-PAGE gels is reported to be around 45-50 kDa.

Antibody Specificity: High

Related Product 1: BCKD-1 blocking peptide

Related Product 2: BCKD-2 pan-specific antibody (Cat. No.: AB-NK257-2)

Related Product 3: BCKD-3 pan-specific antibody (Cat. No.: AB-NK257-3)

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.