Strategies to rescue the consequences of inducible arginase-1 deficiency in mice

Arginase-1 catalyzes the conversion of arginine to ornithine and urea, which is the final step of the urea cycle used to remove excess ammonia from the body. Arginase-1 deficiency leads to hyperargininemia in mice and man with severe lethal consequences in the former and progressive neurological imp...

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Veröffentlicht in:PloS one 2015-05, Vol.10 (5), p.e0125967-e0125967
Hauptverfasser: Ballantyne, Laurel L, Sin, Yuan Yan, St Amand, Tim, Si, Joshua, Goossens, Steven, Haenebalcke, Lieven, Haigh, Jody J, Kyriakopoulou, Lianna, Schulze, Andreas, Funk, Colin D
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Sprache:eng
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Zusammenfassung:Arginase-1 catalyzes the conversion of arginine to ornithine and urea, which is the final step of the urea cycle used to remove excess ammonia from the body. Arginase-1 deficiency leads to hyperargininemia in mice and man with severe lethal consequences in the former and progressive neurological impairment to varying degrees in the latter. In a tamoxifen-induced arginase-1 deficient mouse model, mice succumb to the enzyme deficiency within 2 weeks after inducing the knockout and retain
ISSN:1932-6203
1932-6203
DOI:10.1371/journal.pone.0125967