Animal Models
Studies of the human diseases have benefited from the availability of small laboratory animal models. There is, however, a major handicap for studies involving the endogenous redox systems of primates and other animals - because of the loss of the GULO gene, as a consequence, tissues of other animals generally have high levels of ascorbic acid, which are often only slightly influenced by exogenous vitamin C.
Guinea pigs who also have lost this enzyme function 20 million years ago and depend on dietary vitamin C are often used as a human model.
In 1999, Maeda et al. genetically engineered mice with inactivated GULO gene. The mutant mice, like humans, entirely depend on dietary vitamin C, and they show changes indicating that the integrity of their vasculature is compromised. GULO-/- mice was used as a human model in multiple subsequent studies.
There were number of successful attempts to activate lost enzymatic function in different species.
Read more about this topic: L-gulonolactone Oxidase
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