Deficiency of 2-oxo-glutarate: glyoxylate carboligase activity in primary hyperoxaluria.

Abstract
Activity of 2-oxo-glutarate :glyoxylate carboligase was measured in mitochondrial and cytoplasmic preparations from human liver, spleen, and kidney. The enzyme activity of the cytoplasmic but not of the mitochondrial preparations was markedly reduced in 5 patients with primary hyperoxaluria.