Abstract
An improved radiochemical method to measure the o-aminoJevulinic acid (ALA) synthetase activity in human bone marrow erythroblasts by incubating bone marrow cell homogenate with 14C-succinate and succinyl CoA generating system has been reported. Studies were made to ob tain the optimal conditions for the enzyme assay and to confirm the reliability of the assay pro cedures. ALA-synthetase in erythroblasts was found to be decreased in all 3 cases of iron deficiency anemia and 3 cases of l3-thalassemia minor. In 5 cases of idiopathic sideroblastic anemia, the enzyme activity was markedly decreased. A case of sideroblastic anemia, who restored normal erythropoiesis following the administration of pyridoxal phosphate (PLP) showed low PLP level in serum & blood as well as in vivo evidence of PLP deficiency and an in vitro stimulation of ALA synthetase activity by PLP. Erythroblasts of 4 patients with erythroleukemia showed diminished bone marrow ALA-synthetase activity.
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