Product Name:
BCKD-2
Product Number:
ab-nk257-2
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: Corresponds to amino acid residues I122 to L136
Antibody Modification: Protein kinase pan-specific antibody
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-2 blocking peptide
Related Product 2: BCKD-1 pan-specific antibody (Cat. No.: AB-NK257-1)
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.