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FTCD

Function

Folate-dependent enzyme, that displays both transferase and deaminase activity. Serves to channel one-carbon units from formiminoglutamate to the folate pool.

Binds and promotes bundling of vimentin filaments originating from the Golgi.

Involvement in disease

Glutamate formiminotransferase deficiency

FIGLU-URIA

Autosomal recessive disorder. Features of a severe phenotype, include elevated levels of formiminoglutamate (FIGLU) in the urine in response to histidine administration, megaloblastic anemia, and intellectual disability. Features of a mild phenotype include high urinary excretion of FIGLU in the absence of histidine administration, mild developmental delay, and no hematological abnormalities.

None

The disease is caused by variants affecting the gene represented in this entry.

Pathway

Amino-acid degradation; L-histidine degradation into L-glutamate; L-glutamate from N-formimidoyl-L-glutamate (transferase route): step 1/1.

Sequence Similarities

In the C-terminal section; belongs to the cyclodeaminase/cyclohydrolase family.

In the N-terminal section; belongs to the formiminotransferase family.

Cellular localization

Alternative names

Formimidoyltransferase-cyclodeaminase, Formiminotransferase-cyclodeaminase, LCHC1, FTCD

swissprot:O95954 entrezGene:10841 omim:606806