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N-Acetylglutamate Synthetase (NAGS) Deficiency

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Hepatic Encephalopathy, Hyperammonemia, and Ammonia Toxicity

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 368))

Abstract

N-Acetylglutamate synthetase (NAGS) deficiency is a genetic disorder of ammonia detoxication. These disorders result in acute and chronic hyperammonemia, becoming evident in early infancy. They are associated with a high mortality and morbidity.

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Authors and Affiliations

  1. Dept. of Clinical Chemistry, Inselspital. University of Berne, 3010, Berne, Switzerland

    J. P. Colombo

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  1. J. P. Colombo
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Editors and Affiliations

  1. Instituto de Investigaciones Citologicas de la F. I. B., Valencia, Spain

    Vicente Felipo  & Santiago Grisolia  & 

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Colombo, J.P. (1994). N-Acetylglutamate Synthetase (NAGS) Deficiency. In: Felipo, V., Grisolia, S. (eds) Hepatic Encephalopathy, Hyperammonemia, and Ammonia Toxicity. Advances in Experimental Medicine and Biology, vol 368. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-1989-8_14

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  • DOI: https://doi.org/10.1007/978-1-4615-1989-8_14

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-5820-6

  • Online ISBN: 978-1-4615-1989-8

  • eBook Packages: Springer Book Archive

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