Nutrition and animal models of inherited metabolic disease

Inherited metabolic disease in laboratory animals: a review

“…So, however, must the value of several animal mutants that have been widely used; these are similar, but not homologous, to the comparable human syndromes such as diabetes and dystrophia-muscularis in the mouse (Searle, 1980;Altman and Katz, 1979). Several other mutants and strains have interesting phenotypic features, such as low lipoprotein levels in oedematous mice (Schiffman et al, 1975), diabetes insipidus in SWR mice (Kutscher et al, 1975) and many inherited congenital malformations (Kalter, 1979; see also Bulfield, 1977), but have been unsuccessfully or insufficiently researched to be considered homologues of a human inherited metabolic disease.…”

“…Despite this apparent wealth of material, few of these models are exactly homologous to specific inborn errors of metabolism in man, although some have been transiently induced by nutritional means (Gerritsen and Siegal, 1972;Stephens et al, 1979; see also Hommes, 1979), nor are they readily available in laboratory stocks (Bulfield, 1977(Bulfield, , 1980. It cannot be stressed too strongly that an animal model, to be of value, must be an inherited deficiency of the same enzyme as the one deficient in the human syndrome.…”

“…Most examples of inherited metabolic disease in laboratory animals have turned up by chance (Bulfield, 1977(Bulfield, , 1980Hommes, 1979). There have been a few attempts to screen for animal models using: inbred strains (reviews: Morse, 1978;Festing, 1979;Altman and Katz, 1979); phenotypically unusual mutants (Kacser et al, 1973;De Mars et al, 1976;Eicher et al, 1976;Hutton and Bernstein, 1973); spontaneous (Pivetta and Green, 1973) or induced mutations (Feinstein et al, 1964(Feinstein et al, , 1966Gluecksohn-Waelsch et al, 1974;Poppet al, 1979); and wild-caught animals (Padua et al, 1978;Bulfield and Moore, 1978;Berry, 1980).…”