Product Name:
BCKD-1
Product Number:
ab-nk257-1
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: BCKDK protein kinase pan-specific antibody
Antibody Code: NK257-1
Antibody Target Type: Pan-specific
Protein UniProt: O14874
Protein SigNET: BCKD
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: This antibody detects the target protein in the following species due to conservation of amino acid sequence: Human | Chimpanzee | Rhesus macaque | Rat | Mouse | Zebra fish.
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: BCKDK (56-70) antibody blocking peptide (Cat. No.: PE-01AZT85)
Related Product 2: BCKD expression antibody (Cat. No.: AB-NK257-2)
Related Product 3: BCKD expression antibody (Cat. No.: AB-NK257-3)
Scientific Background: BCKD (BCKDK) is a protein-serine/threonine kinase of the Atypical group and PDHK family. It belongs to the mitochondrial protein kinases family, which is similar to histidine kinases, though it primarily acts as a serine/threonine kinase. It is associated with the mitochondrial matrix and features a HATPase_c domain. It forms a homodimer (or sometimes a homotetramer) and binds to the E2 core of the BCKD complex. BCKD dimerizes through direct interaction of two opposing nucleotide-binding domains. It also features a four-helix bundle domain. It phosphorylates (at Ser-347) and inactivates the E1-alpha subunit of the branched-chain ketoacid dehydrogenase (BCKDK), which is involved in the catabolism of valine, leucine and isoleucine. By inactivating the BCKD complex, this kinase prevents the over-oxidation of branched-chain amino acids (BCAAs), thereby conserving them for protein synthesis. High levels of branched-chain alpha-ketoacids (BCKAs), such as alpha-ketoisocaproate, act as inhibitors of BCKDK, allowing the BCKD complex to remain active and break down excess BCAAs. Deficiencies or mutations in the BCKDK gene can lead to a condition known as BCKDK deficiency or BCAA depletion syndrome, which is associated with autism spectrum disorder, intellectual disability, epilepsy, and microcephaly. The syndrome may respond to dietary supplementation. Low levels of BCKD expression are associated with pathologic muscular conditions and decreased muscle integrity. Elevated expression of BCKDK has been linked to increased malignancy in several cancers (including colorectal, ovarian, and lung), where it acts to promote tumour proliferation and metastasis. This description may include information annotated by UniProt and/or Google AI.