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Mouse model for human arginase deficiency.

Ramaswamy K Iyer1, Paul K Yoo, Rita M Kern

  • 1Department of Pathology and Laboratory Medicine and the Mental Retardation Research Center, University of California Los Angeles School of Medicine, Los Angeles, California 90095-1732, USA. riyer@mednet.ucla.edu

Molecular and Cellular Biology
|June 8, 2002
PubMed
Summary
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Liver arginase (AI) deficiency causes hyperargininemia. AI knockout mice showed hyperammonemia and died young, mimicking human disease and offering a model for treatment research.

Area of Science:

  • Biochemistry
  • Genetics
  • Pathology

Background:

  • Liver arginase (AI) deficiency leads to hyperargininemia, a condition marked by neurological issues and hyperammonemia.
  • The human condition presents with progressive mental impairment, growth retardation, spasticity, and life-threatening hyperammonemia episodes.

Purpose of the Study:

  • To create a mouse model for studying hyperargininemia and its associated pathology.
  • To investigate the pathobiological consequences of complete arginase deficiency in a mammalian model.

Main Methods:

  • Homologous recombination was used to generate a knockout mouse strain lacking functional arginase AI.
  • Phenotypic analysis included monitoring survival, plasma ammonia levels, liver histology, and plasma amino acid profiling.

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Main Results:

  • Arginase AI knockout mice exhibited no liver arginase activity, severe hyperammonemia, and mortality between postnatal days 10-14.
  • Elevated plasma arginine and ammonia, altered proline and ornithine levels, and hepatocyte abnormalities were observed in knockout mice.
  • Changes in glutamic acid, citrulline, histidine, and branched-chain amino acid levels were also noted.

Conclusions:

  • The arginase AI-deficient mouse accurately models key aspects of human hyperargininemia.
  • This mouse model is valuable for elucidating disease mechanisms and testing therapeutic strategies, including pharmaceutical interventions and gene therapy.