Formiminotransferase activity of liver from mice with pyridoxine deficiency.

Recently we have encountered an infant of formiminotransferase deficiency syndrome associated with megaloblastic pyridoxine responsive anemia of congenital origin.1 In 1966 Vitale et al.2 reported that formiminotransferase activity of the liver was markedly decreased in rats with iron deficiency and suggested a possibility of requirement of iron for function and/or formation of formiminotransferase system. In 1964 Harris3 reported that pyridoxine took an important role in heme biosynthesis in two adult cases of megaloblastic pyridoxine responsive anemia in such a way that pyridoxine was necessary for release of heme from mitochondria of reticulocytes from these patients. On taking these results into consideration in respect to the association of formiminotransferase deficiency with megaloblastic-pyridoxine responsive anemia of our patient, a question may arise whether or not an impaired utilization of iron due to pyridoxine dependency or deficiency would be a causative factor common to both a defective activity of formiminotransferase and pyridoxine-responsive anemia in our own patient. The aim of the present investigation is to determine whether or not pyridoxine deficiency would exert an effect, if any, upon formiminotransferase activity of the liver of mice.