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A protocol for the successful long‐term enzyme replacement therapy of scurvy in guinea pigs
Author(s) -
Hadley K.,
Sato P.
Publication year - 1988
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01800427
Subject(s) - ascorbic acid , scurvy , guinea pig , enzyme , vitamin c , vitamin , oxidase test , medicine , biology , ascorbic acid deficiency , biochemistry , endocrinology , food science
Summary Gulonolactone oxidase, a key enzyme in the biosynthesis of ascorbic acid, is missing from guinea pigs and certain other scurvy‐prone species. Weekly intraperitoneal injections of glutaraldehyde cross‐linked immunoprecipitates of this enzyme have been shown to provide guinea pigs with the capability of synthesizing their own ascorbic acid and of surviving without an exogenous source of this vitamin. This protocol, however, was successful in only a small percentage of the animals tested. The reasons for the limited therapeutic success were investigated. Apparently, the gulonolactone oxidase‐treated guinea pigs fed without ascorbic acid were receiving insufficient nutrition. By supplementing these enzyme‐treated animals with vitamins A, B, D and E and selenium, we successfully maintained a high proportion of guinea pigs fed without vitamin C.