| knockout mice exhibited severe hyperammonemia and died between postnatal days 10 and 14 | |
livers of Arg1-deficient animals showed hepatocyte abnormalities, including cell swelling and inclusion. The mean arginine level in plasma showed of Arg1-knockout mice was 4-fold and 3-fold greater than in wildtype and heterozygous mice, respectively and proline and ornithine levels were reduced |
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Arg1-KO mice duplicate several pathobiologic aspects of human argininemia |
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Arg1/Arg2 double-knockout mice exhibited the phenotype of the Arg1 knockout mice, with the additional absence of Arg2 not exacerbating the phenotype. Arginine levels increased roughly 100-fold and ornithine decreased roughly 10-fold compared with wildtype |
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alpha-keto-delta-guanidinovaleric acid, alpha-N-acetylarginine and argininic acid are increased in brain tissue from Arg1 KO and Arg1/Arg2 double-KO mice |