DBT : dihydrolipoamide branched chain transacylase E2


Description

The DBT (dihydrolipoamide branched chain transacylase E2) is a protein-coding gene located on chromosome 1.

The DBT gene provides instructions for making part of a group of enzymes called the branched-chain alpha-keto acid dehydrogenase (BCKD) enzyme complex. Specifically, the protein produced from the DBT gene forms a critical piece of the enzyme complex called the E2 component. The BCKD enzyme complex is responsible for one step in the normal breakdown of three protein building blocks (amino acids). These amino acids—leucine, isoleucine, and valine—are obtained from the diet. They are present in many kinds of food, particularly protein-rich foods such as milk, meat, and eggs. The BCKD enzyme complex is active in mitochondria, which are specialized structures inside cells that serve as energy-producing centers. The breakdown of leucine, isoleucine, and valine produces molecules that can be used for energy.

The DBT gene encodes a protein that is part of the branched-chain alpha-keto acid dehydrogenase complex (BCKDH), which is responsible for breaking down certain amino acids. The protein acts as the E2 component of the complex, which is responsible for accepting and transferring acyl groups to coenzyme A. This process is crucial for the overall conversion of alpha-keto acids to acyl-CoA and CO2.

DBT is also known as BCATE2, BCKAD-E2, BCKADE2, BCKDH-E2, BCOADC-E2, E2, E2B.

Associated Diseases


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