Rabbit anti-Human, Mouse BCKDK Polyclonal Antibody | anti-BCKDK antibody

BCKDK, CT (T340) (3-methyl-2-oxobutanoate Dehydrogenase Lipoamide Kinase, Mitochondrial, Branched-chain alpha-ketoacid Dehydrogenase Kinase, BCKDHKIN, BCKD-kinase) (Azide free) (HRP)

Cat. Num. AAA6465002

• Gene Names: BCKDK; BDK; BCKDKD
• Reactivity: Human, Mouse
• Applications: Western Blot, ELISA
• Purity: Purified by Protein G Affinity Chromatography.
• Synonyms: BCKDK; Polyclonal Antibody; CT (T340) (3-methyl-2-oxobutanoate Dehydrogenase Lipoamide Kinase; Mitochondrial; Branched-chain alpha-ketoacid Dehydrogenase Kinase; BCKDHKIN; BCKD-kinase) (Azide free) (HRP); anti-BCKDK antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human BCKDK. Species Crossreactivity: mouse.
• Purity/Purification: Purified by Protein G Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Horseradish Peroxidase (HRP).

• Applicable Applications for anti-BCKDK antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:100-1:500; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the C-terminal region of human BCKDK (KLH).
• Conjugate: HRP
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(The anti-BCKDK Pab is used in Western blot to detect BCKDK in mouse intestine tissue lysate (Lane 1) and Hela cell lysate (Lane 2).)

Western Blot (WB)

(Western blot analysis of BCKDK (arrow) using rabbit polyclonal BCKDK Antibody (C-term T340) (RB05257).293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected with the BCKDK gene (Lane 2) (Origene Technologies).)

Product Categories/Family for anti-BCKDK antibody

Antibodies; Protein Kinase Substrates

NCBI and Uniprot Product Information

• NCBI GI #: 171906593
• NCBI GeneID: 10295
• NCBI Accession #: NP_001116429.1
• NCBI GenBank Nucleotide #: NM_001122957.2
• UniProt Accession #: O14874; Q6FGL4; Q96IN5
• Molecular Weight: 46,360 Da
• NCBI Official Full Name: [3-methyl-2-oxobutanoate dehydrogenase
• NCBI Official Synonym Full Names: branched chain ketoacid dehydrogenase kinase
• NCBI Official Symbol: BCKDK
• NCBI Official Synonym Symbols: BDK; BCKDKD
• NCBI Protein Information: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; BCKDHKIN; BCKD-kinase; branched chain alpha-ketoacid dehydrogenase kinase
• UniProt Protein Name: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
• Protein Family: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase
• UniProt Gene Name: BCKDK
• UniProt Synonym Gene Names: BCKD-kinase; BCKDHKIN
• UniProt Entry Name: BCKD_HUMAN

NCBI Description

The branched-chain alpha-ketoacid dehydrogenase complex (BCKD) is an important regulator of the valine, leucine, and isoleucine catabolic pathways. The protein encoded by this gene is found in the mitochondrion, where it phosphorylates and inactivates BCKD. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Dec 2012]

Uniprot Description

BCKDK: an atypical protein kinase associated with the mitochondrial matrix. Contains a HATPase_c domain, found in several ATP-binding proteins including protein histidine kinases (PHKs), PHDKs, DNA gyrase B, topoisomerases, heat shock proteins, and DNA mismatch repair proteins. Unlike PHKs, BCK dimerizes through direct interaction of two opposing nucleotide-binding domains. Phosphorylates and inactivates the E1-alpha subunit of the branched-chain ketoacid dehydrogenase (BCKDK), the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Inactivating mutations have been discovered in BCKDK in families with autism, epilepsy, and intellectual disability, a syndrome that may respond to dietary supplementation.

Protein type: Protein kinase, atypical; Kinase, protein; Mitochondrial; EC 2.7.11.4; ATYPICAL group; PDHK family

Chromosomal Location of Human Ortholog: 16p11.2

Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrion

Molecular Function: protein serine/threonine kinase activity; protein binding; [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity; kinase activity; ATP binding

Biological Process: branched chain family amino acid catabolic process; amino acid catabolic process; phosphorylation; protein amino acid phosphorylation

Disease: Branched-chain Ketoacid Dehydrogenase Kinase Deficiency