ON THE RENAL TUBULAR DAMAGE IN HEREDITARY TYROSINEMIA AND ON THE FORMATION OF SUCCINYLACETOACETATE AND SUCCINYLACETONE1

摘要: ABSTRACT. Fallstrom, S.-P., Lindblad, B. and Steen, G. (Department of Paediatrics, East Hospital, University Gothenburg, Department Molndal's Molndal, Clinical Chemistry, Sweden). On the renal tubular damage in hereditary tyrosinemia on formation succinylacetoacetate succinylacetone. Acta Paediatr Scand, 70:315, 1981.–Phenylalanine homogentisate increase concentration succinylacetone both serum urine patients with therefore excretion 5-aminolevulinate. Both phenylalanine cause a proteinuria which is agreement our hypothesis that their metabolites maleylacetoace-tate fumarylacetoacetate are toxic compounds tyrosinemia. The patient highest has slightest whereas one lowest these more pronounced proteinuria. It suggested this caused by difference ability to reduce presumed maleylacetoacetate, i.e. precursors succinylacetoacetate.