Glutamate formiminotransferase

Malvagia S, la Marca G, Casetta B, et al (2006) Falsely elevated C4-carnitine as expression of glutamate formiminotransferase deficiency in tandem mass spectrometry newborn screening. J Mass Spectrom 41 263-265... 

Similarly, a deficiency of glutamate formiminotransferase leads to accumulation of Figlu and high levels of serum folate. 

Formiminoglutamic aciduria (congenital) Type r Formiminoglutamic acid, hydantoin-5-propionic acid Glutamate formiminotransferase (EC 2.1.2.5) Unproven 16.5... 

Formylglutamate can transfer its formyl group directly onto tetrahydrofolate to yield 5-formyl-tetrahydrofolate. Formyl-glutamate is not a normal physiological intermediate, and the formation of 5-formyl-tetrahydrofolate is probably a side reaction of FIGLU formiminotransferase. 

Glycine formiminotransferase catalyzes the reaction of formiminoglycine (XCVIII) and tetrahydrofolate (XCIX) to glycine (C) and 5-formiminotetrahydro-folate (Cl) (R is benzoyl-L-glutamate). Uyeda and Rabinowitz (1965) have purified... 

When a crude liver extract is added to the purified urocanase, decomposition of urocanic acid proceeds to W-formimino-L-glutamic acid. The enzyme carrying out this stage of the reaction has been named imidazolonepropionic acid hydrolase. In view of the fact that im-idazolonepropionic acid has not been isolated and study of the hydrolysis reaction can only be performed in incubation mixtures in which urocanic acid is reacted with purified urocanase very little is known about the properties of the hydrolase enzyme. This enzyme has been purified about tenfold by ammonium sulfate fractionation in our laboratory (266). The enzyme activity is shown by an acceleration in the rate of the decomposition of the 264 my absorption. The product formed was shown to be formimino glutamic acid by testing with purified formiminotransferase which is specific for this compound. 

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