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Studies on Ascorbic Acid Related to the Genetic Basis of Scurvy

1979; Elsevier BV; Linguagem: Inglês

10.1016/s0083-6729(08)60981-4

2162-2620

Paul H. Sato, Sidney Udenfriend,

Alcoholism and Thiamine Deficiency

This chapter presents the studies on ascorbic acid related to the genetic basis of scurvy. The importance of ascorbic acid has long been known, scurvy being the first recognized deficiency disease in humans. The vitamin has been shown to be involved in biological hydroxylation reactions of a number of metabolic pathways. Studies with radioactive tracers have shown that ascorbic acid is derived from glucose as a part of the glucuronic acid pathway. It is found that in the presence of hydroxylamine or semicarbazide L-gulonolactone oxidase, which had been purified nearly to homogeneity, converted D-glucuronolactone to ascorbic acid. The final step in the pathway from glucose to ascorbic acid is catalyzed by L-gulonolactone oxidase. The requirement for ascorbic acid by some mammals, results from an inherited lack of an active form of this enzyme. Sulfhydryl reducing agents have been found to stimulate L-gulonolactone oxidase activity of purified rat and goat enzyme.