the findings of decreased uroporphyrinogen synthesis without an increase in ala synthetase in mitochondria-containing cells from subjects with acute intermittent porphyria are compatible with the concept that defective pbg ultilization is the fundamental defect in heme biosynthesis in this disease and the possibility that ala synthetase is `` '' irreversibly '' '' repressed in nonhepatic tissues .
C0026237
mitochondria
C0007634
cells