Rabbit BCKDH Polyclonal Antibody | anti-BCKDH antibody
BCKDH Antibody FITC-Conjugated
ELISA: 1:10,000
Western Blot: 1:500
Catalyzes phosphorylation and inactivation of branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine.
NCBI and Uniprot Product Information
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; EC 2.7.11.4; Kinase, protein; Mitochondrial; ATYPICAL group; PDHK family
Chromosomal Location of Human Ortholog: 16p11.2
Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrion
Molecular Function: [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity; ATP binding; kinase activity; protein binding; protein serine/threonine kinase activity
Biological Process: amino acid catabolic process; branched chain family amino acid catabolic process; phosphorylation; protein amino acid phosphorylation
Disease: Branched-chain Ketoacid Dehydrogenase Kinase Deficiency
Research Articles on BCKDH
Similar Products
Product Notes
The BCKDH bckdk (Catalog #AAA541041) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The BCKDH Antibody FITC-Conjugated reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's BCKDH can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB). Dot Blot: 1:10,000 ELISA: 1:10,000 Western Blot: 1:500. Researchers should empirically determine the suitability of the BCKDH bckdk for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "BCKDH, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
If you are ready to order, navigate to Shopping Cart and get ready to checkout.
