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

BCKDK, ID (3-methyl-2-oxobutanoate Dehydrogenase Lipoamide Kinase, Mitochondrial, Branched-chain alpha-ketoacid Dehydrogenase Kinase, BCKDHKIN, BCKD-kinase) (MaxLight 550)

Cat. Num. AAA6465016

• Gene Names: BCKDK; BDK; BCKDKD
• Reactivity: Human
• Applications: Immunohistochemistry, Western Blot
• Purity: Purified by Ammonium Sulfate Precipitation.
• Synonyms: BCKDK; Polyclonal Antibody; ID (3-methyl-2-oxobutanoate Dehydrogenase Lipoamide Kinase; Mitochondrial; Branched-chain alpha-ketoacid Dehydrogenase Kinase; BCKDHKIN; BCKD-kinase) (MaxLight 550); anti-BCKDK antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human BCKDK.
• Purity/Purification: Purified by Ammonium Sulfate Precipitation.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight550.

• Applicable Applications for anti-BCKDK antibody: Immunohistochemistry (IHC), FLISA, Western Blot (WB)
• Application Notes: IHC: 1:50-1:100; FLISA: 1:1,000; WB: 1:100-1:500; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the central region of human BCKDK (KLH).
• Conjugate: MaxLight550
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight550 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

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

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human cancer tissue reacted with the primary antibody, which was peroxidase-conjugated to the secondary antibody, followed by AEC staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated. BC = breast carcinoma; HC = hepatocarcinoma.)

Product Categories/Family for anti-BCKDK antibody

Antibodies; Protein Kinase Substrates

References

1. Chang, C.F., et al., J. Biol. Chem. 277(18):15865-15873 (2002). 2. Popov, K.M., et al., J. Biol. Chem. 267(19):13127-13130 (1992). 3. Zneimer, S.M., et al., Genomics 10(3):740-747 (1991).

NCBI and Uniprot Product Information

• NCBI GI #: 171906589
• NCBI GeneID: 10295
• NCBI Accession #: NP_005872
• NCBI GenBank Nucleotide #: NM_005881.3
• UniProt Accession #: O14874; Q6FGL4; Q96G95; Q96IN5; A8MY43
• Molecular Weight: 41,090 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
• 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