Familial Occurrence of Formiminotransferase Deficiency Syndrome

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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 formiminotransfe...

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Formiminotransferase deficiency syndrome associated with megaloblastic anemia responsive to pyridoxine or folic acid.

Formiminotransferase deficiency syndrome1,2 was firstly discovered by us as a new inborn error of folate metabolism which was characterized by 1) mental retardation, 2) hyperfolic acidemia, and 3) an excessive urinary excretion of formiminoglutamic acid (FIGLU) following an oral dose of L-histidine. A definite diagnosis of this syndrome was established by demonstrating a defective activity of f...

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ژورنال

عنوان ژورنال: The Tohoku Journal of Experimental Medicine

سال: 1968

ISSN: 0040-8727,1349-3329

DOI: 10.1620/tjem.96.211