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. Author manuscript; available in PMC: 2020 Nov 1.
Published in final edited form as: J Inherit Metab Dis. 2019 Mar 5;42(6):1044–1053. doi: 10.1002/jimd.12048

Figure 2. Characteristics of Neonatal CPS1-Deficient Mice.

Figure 2.

A) The genotypes are distributed among 104 progeny of CPS1 matings of heterozygote mice; B) Western blot showing hepatic CPS1 protein in wild type (+/+), heterozygote (+/−) and knockouts (−/−); representative immunostaining for CPS1 shows hepatic CPS1 expression in liver in Cps1+/+ (C) and Cps1+/− (D) mice with absent expression in knockout mouse liver (E); (F) CPS1 activity in hepatic extracts from Cps1+/+, Cps1+/− and knockout mice correspond with that of CPS1-specific mRNA (G); plasma ammonia levels of neonatal Cps1+/+, Cps1+/− and knockout mice (n=5 per group; except in the CPS1 enzyme assay [n=3 Cps1+/−, n=4 Cps1+/+ and n=5 Cps1−/−]). (Data are presented as mean ± standard deviation.) (CP = Carbamoyl Phosphate).