Spontaneous Animal Models of Ornithine Transcarbamylase Deficiency: Studies on Serum and Urinary Nitrogenous Metabolites

摘要: Various groups of spf (sparse fur) and ash (allele with abnormal skin hair) mice, were compared in respect their liver ornithine transcarbamylase (OTC) activity, urinary orotate concentrations serum NH3 glutamine. While OTC activity was comparable both the strains, excretion lower spfash mice. In contrast to females, ash/+ heterozygotes cannot be distinguished from normal +/+ as has no significant correlation activity. However, glutamine is significantly correlated deficiency strains. Administration 1% sodium benzoate spf/Y males resulted a pattern hippurate urea N similar clinical trials humans. Urinary reduced, while ad lib dietary intake increased almost 3 fold. Both spontaneous animal models have considerable potential used investigation etiology, specific pathology nutritional therapy congenital hyperammonemias.