AMT Activators

Aminomethyltransferase (AMT) is an essential enzyme involved in the catabolism of amino acids and the formation of the amino acid glycine. Encoded by the AMT gene in humans, this protein is part of the glycine cleavage system (GCS), a multi-enzyme complex localized in the mitochondria.The primary function of AMT is to transfer a methylamine group from the tetrahydrofolate-bound substrate to a lipoate cofactor. This process is an essential step in the metabolism of glycine, converting it into carbon dioxide and ammonia, which can then enter central metabolic pathways.AMT is a homotrimeric protein, which means it exists as a complex of three identical subunits. This trimeric structure is important for the protein's enzymatic function and stability.

Mutations in the AMT gene can lead to a disorder known as glycine encephalopathy, previously known as nonketotic hyperglycinemia. This condition is characterized by abnormally high levels of glycine in bodily fluids and tissues, particularly the brain. Indicators may include intellectual disability, seizures, hypotonia, and breathing difficulties. The condition can be severe, often appearing in the neonatal period, and can be life-threatening if not managed appropriately.In conclusion, AMT plays a critical role in amino acid metabolism. Its importance is underscored by the severe consequences of its malfunction, as seen in glycine encephalopathy, and its emerging roles in cancer and immunity highlight the need for continued research into this protein.