Western Blot (WB)
(WB Suggested Anti-DBT Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:1562500Positive Control: HT1080 cell lysateThere is BioGPS gene expression data showing that DBT is expressed in HT1080)
Rabbit DBT Polyclonal Antibody | anti-DBT antibody
DBT antibody - N-terminal region
Western Blot (WB)
(WB Suggested Anti-DBT Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:1562500Positive Control: HT1080 cell lysateThere is BioGPS gene expression data showing that DBT is expressed in HT1080)
Western Blot (WB)
(WB Suggested Anti-DBT Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:1562500Positive Control: HT1080 cell lysateThere is BioGPS gene expression data showing that DBT is expressed in HT1080)
Target Description: The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.
NCBI and Uniprot Product Information
NCBI Description
The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined. [provided by RefSeq, Jul 2008]
Uniprot Description
DBT: 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 DBT are the cause of maple syrup urine disease type 2 (MSUD2). MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine. Belongs to the 2-oxoacid dehydrogenase family.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 2.3.1.168; Mitochondrial; Transferase
Chromosomal Location of Human Ortholog: 1p31
Cellular Component: mitochondrion; mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix
Molecular Function: dihydrolipoyllysine-residue (2-methylpropanoyl)transferase activity
Biological Process: branched chain family amino acid catabolic process
Disease: Maple Syrup Urine Disease
Research Articles on DBT
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Product Notes
The DBT dbt (Catalog #AAA3210550) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The DBT antibody - N-terminal region reacts with Cow, Dog, Guinea Pig, Horse, Human, Mouse, Rabbit, Rat, Yeast, Zebrafish and may cross-react with other species as described in the data sheet. AAA Biotech's DBT can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunohistochemistry (IHC). Researchers should empirically determine the suitability of the DBT dbt 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. The amino acid sequence is listed below: Synthetic peptide located within the following region: NYVCFFGYPS FKYSHPHHFL KTTAALRGQV VQFKLSDIGE GIREVTVKEW. It is sometimes possible for the material contained within the vial of "DBT, 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
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