L-gulono-gamma-lactone oxidase deficiency in rats with osteogenic disorder: enzymological and immunochemical studies

A strain of Wistar rats with L-gulono-gamma-lactone oxidase deficiency has recently been established. The activity of L-gulono-gamma-lactone oxidase in hepatic microsomes of the mutant rats was not detectable, while that of heterozygous rats was about half that of normal rats. These results were confirmed by immunological quantitation using antibody directed against this enzyme. Thus, it appears that these mutant rats either possess no enzyme at all or a very aberrant form of the enzyme in the liver. Aldonolactonase, another enzyme participating in the biosynthesis of L-ascorbic acid, was found to be present in normal amount in the mutant strain.