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formimidoyltransferase-cyclodeaminase (formiminotransferase-cyclodeaminase, FTCD, LCHC1 [includes: glutamate formimidoyltransferase/glutamate formiminotransferase/glutamate formyltransferase & formimidoyltetrahydrofolate cyclodeaminase/formiminotetrahydrofolate cyclodeaminase])

Function: 1) folate-dependent enzyme 2) displays both transferase & deaminase activity 3) channels one-carbon units from formiminoglutamate to folate pool 4) histidine degradation, 4th & steps 5) may be produced at very low levels due to a premature stop codon in the mRNA, leading to nonsense-mediated mRNA decay Cofactor: pyridoxal phosphate Structure: 1) homooctamer 2) subunits are arranged as a tetramer of dimers, & form a planar ring-shaped structure 4) polyglutamate binding sites Compartment: cytoplasm Alternative splicing: named isoforms=4 Note: - may be produced at very low levels due to a premature stop codon in the mRNA, leading to nonsense-mediated mRNA decay Pathology: - defects in FTCD are the cause of formiminoglutamicaciduria

General

enzyme

Properties

SIZE: MW = 59 kD entity length = 541 aa COMPARTMENT: cytoplasm MOTIF: Formiminotransferase {1-236} MOTIF: histidine residue {H82} Folate binding (Potential) {163-172}

Database Correlations

OMIM correlations MORBIDMAP 606806 UniProt O95954 PFAM correlations ENZYME correlations

References

UniProt :accession O95954