Typical Testing Data
Human Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial ELISA Kit | DBT elisa kit
Human Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial ELISA Kit
Typical Testing Data
Typical Testing Data
Typical Standard Curve
Typical Standard Curve
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: Transferase; EC 2.3.1.168; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation
Chromosomal Location of Human Ortholog: 1p31
Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrion
Molecular Function: dihydrolipoyllysine-residue (2-methylpropanoyl)transferase activity; ubiquitin protein ligase binding
Biological Process: branched chain family amino acid catabolic process; glyoxylate metabolic process
Disease: Maple Syrup Urine Disease
Research Articles on DBT
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Product Notes
The Human DBT dbt (Catalog #AAA761050) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA761050 ELISA Kit recognizes Human DBT. It is sometimes possible for the material contained within the vial of "Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial, ELISA Kit" 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
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