branched-chain amino acid biosynthetic process

Definition

Target type: biologicalprocess

The chemical reactions and pathways resulting in the formation of amino acids containing a branched carbon skeleton, comprising isoleucine, leucine and valine. [GOC:ai]

Branched-chain amino acid biosynthesis is a fundamental metabolic process that produces essential amino acids valine, leucine, and isoleucine. These amino acids are crucial for protein synthesis, muscle growth, and various other metabolic functions. The biosynthesis pathway is complex and involves multiple steps, each catalyzed by specific enzymes.

The starting point for branched-chain amino acid biosynthesis is pyruvate, a key intermediate in carbohydrate metabolism. Pyruvate is first converted to α-ketoisovalerate, which is then transaminated to valine. Leucine biosynthesis begins with α-ketoisocaproate, derived from α-ketoisovalerate. Isoleucine biosynthesis starts with α-keto-β-methylvalerate, formed from threonine and pyruvate.

The biosynthesis of valine, leucine, and isoleucine involves a series of enzymatic reactions, including:

* **Transamination:** Transfer of an amino group from an amino acid to a keto acid.
* **Decarboxylation:** Removal of a carboxyl group (COOH) from a molecule.
* **Reduction:** Addition of hydrogen atoms to a molecule.
* **Isomerization:** Rearrangement of atoms within a molecule.

The enzymes involved in branched-chain amino acid biosynthesis are highly regulated, ensuring that these essential amino acids are produced in appropriate amounts. Deficiencies in these enzymes can lead to various metabolic disorders.

Branched-chain amino acid biosynthesis is an essential process for all living organisms. It is tightly regulated to meet the needs of the organism for these essential amino acids. This intricate process highlights the complex and interconnected nature of metabolism.'
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Proteins (2)

Compounds (2)