Testing Data
Rabbit BCKDHB Polyclonal Antibody | anti-BCKDHB antibody
BCKDHB Antibody
IHC: 1:20-1:200
Testing Data
Testing Data
NCBI and Uniprot Product Information
NCBI Description
Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform. [provided by RefSeq, Jul 2008]
Uniprot Description
BCKDHB: The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Defects in BCKDHB are the cause of maple syrup urine disease type IB (MSUD1B). MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; EC 1.2.4.4; Oxidoreductase
Chromosomal Location of Human Ortholog: 6q14.1
Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrion; mitochondrial matrix
Molecular Function: protein binding; alpha-ketoacid dehydrogenase activity; protein complex binding; carboxy-lyase activity; 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity
Biological Process: response to cAMP; response to glucocorticoid stimulus; branched chain family amino acid catabolic process; response to nutrient
Disease: Maple Syrup Urine Disease
Research Articles on BCKDHB
Similar Products
Product Notes
The BCKDHB bckdhb (Catalog #AAA2520686) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The BCKDHB Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's BCKDHB can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC). WB: 1:200-1:2000 IHC: 1:20-1:200. Researchers should empirically determine the suitability of the BCKDHB bckdhb 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 "BCKDHB, 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.
