Beneficial Effect of Orotic Acid upon Galactose Metabolism of Erythrocytes from Congenital Galactosemia

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Abstract
The effect of orotic acid on galactose metabolism in patients with congenital galactosemia was investigated in vitro using blood samples. During the anerobic incubation with galactose, the galactosemic erythrocytes showed a lower production of lactic acid, a remarkable increase of hexose-phosphate and an increased accumulation of isotopic P in hexose-phosphate fraction. These abnormal findings in galactosemic erythrocytes were obviously corrected by the addition of orotic acid or uridine to the incubation mixture. This beneficial effect of orotic acid upon galactose metabolism in vitro in galactosemic erythrocytes is ascribed to an enhancement of the accessory pathway via UDPGal pyrophosphorylase by UTP derived from orotic acid added.