glycine cleavage system T protein; aminomethyltransferase (GCVT, AMT GCST)

Function

• component of glycine cleavage system

[protein]-S(8)-aminomethyldihydrolipoyllysine + tetrahydrofolate
              <-->
[protein]-dihydrolipoyllysine + 5,10-methylenetetrahydrofolate + NH3

Structure

belongs to the gcvT family

Compartment

mitochondria

Pathology

• defects in AMT are a cause of non-ketotic hyperglycinemia

More general terms

• mitochondrial protein
• aminotransferase

Component of

• glycine cleavage system

Database

• UniProt: http://www.uniprot.org/uniprot/P48728.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=275
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:275
• OMIM: https://mirror.omim.org/entry/238310
• OMIM: https://mirror.omim.org/entry/605899