Formimidoylglutamase

Formimidoylglutamase (also known as FIGLU transferase) is an enzyme that plays a crucial role in the metabolism of amino acids. It is specifically involved in the metabolic pathway of histidine, a vital amino acid for human health.

Function

Formimidoylglutamase catalyzes the hydrolysis of N-formimidoyl-L-glutamate to L-glutamate and formamide. This reaction is the final step in the metabolism of histidine, converting the formimidoyl group from histidine into formate and ammonia, which can be excreted from the body.

Clinical significance

Deficiency in formimidoylglutamase can lead to a rare metabolic disorder known as Formiminoglutamic aciduria (FIGLU-uria). This condition is characterized by the accumulation of formiminoglutamic acid (FIGLU) in the urine, especially after the ingestion of histidine. Symptoms can include developmental delay, intellectual disability, and megaloblastic anemia.

Structure and location

Formimidoylglutamase is a cytosolic enzyme, meaning it is found in the cytosol of cells. It is primarily expressed in the liver, where it plays a key role in amino acid metabolism.

See also

• Histidine metabolism
• Formiminoglutamic aciduria
• Enzyme

References

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