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Aminotransferase and related amino acid-metabolizing enzymes

Molecular classification
Enzyme, Transferase, Pyridoxal phosphate-dependent enzyme
01

Overview

Aminotransferases and related amino acid-metabolizing enzymes represent a diverse superfamily of proteins, predominantly pyridoxal 5-phosphate (PLP)-dependent, that facilitate the transfer of amino groups between amino acids and alpha-keto acids (UniProt). These enzymes are central to nitrogen homeostasis, linking protein metabolism to the tricarboxylic acid (TCA) cycle and gluconeogenesis (StatPearls). In clinical practice, specific members such as 4-aminobutyrate aminotransferase (GABA-T) are therapeutic targets; for example, the drug vigabatrin inhibits GABA-T to increase GABA levels in the brain for the treatment of refractory epilepsy (FDA). Furthermore, enzymes like alanine aminotransferase (ALT) and aspartate aminotransferase (AST) are gold-standard biomarkers for detecting liver and muscle damage (NIH). Beyond metabolic and neurological roles, these enzymes are increasingly recognized for their contribution to the metabolic flexibility of cancer cells, making them subjects of interest for novel anti-tumor strategies (Nature Reviews Cancer). However, because these enzymes are involved in fundamental metabolic pathways, pharmacological intervention requires careful management to avoid systemic toxicity or metabolic imbalances.

Other names
TransaminasesAmino acid metabolizing enzymesPLP-dependent aminotransferasesAmino acid transferases
02

Mechanism of action

Inhibition of specific enzyme activity to modulate the concentration of signaling molecules (e.g., increasing GABA via GABA-T inhibition) or to disrupt metabolic pathways essential for disease progression (StatPearls, PubMed).

03

Biological functions

Amino acid metabolismNitrogen homeostasisNeurotransmitter synthesis and degradationGluconeogenesisUrea cycle regulationCarbon-nitrogen balance
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Disease associations

EpilepsyLiver diseaseCancerNeurological disordersMetabolic disordersHepatocellular injury
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Safety considerations

HepatotoxicitySystemic metabolic disruptionPermanent visual field defects (associated with Vigabatrin)Neurological side effects
06

Interacting drugs

Vigabatrin

3 more in the full profile.

07

Biomarkers

Alanine aminotransferase (ALT) serum levelsAspartate aminotransferase (AST) serum levelsGamma-aminobutyric acid (GABA) levels

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