HEREDITARY-DEFICIENCY OF ADENINE PHOSPHORIBOSYL TRANSFERASE

  • 1 January 1980
    • journal article
    • research article
    • Vol. 15  (3) , 233-238
Abstract
A complete deficiency (> 0.02%) of the enzyme adenine phosphoribosyl transferase (APRT; EC 2.4.2.8) is described in a 12-yr-old patient. The patient developed acute renal failure at the age of 18 mo. due to radiolucent uric acid stones. Using high pressure liquid chromatography, 2,8-dihydroxyadenine was found in the urine rather than uric acid. The pedigree indicates an autosomal recessive mode of inheritance of this disorder.