ArticlePloS one2013
Inducible arginase 1 deficiency in mice leads to hyperargininemia and altered amino acid metabolism.
Article in PloS one, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 48 citations in OpenAlex.
- Pharmacological and genetic inhibition of ARG2 in CXCR2Theranostics · 2025Article
- Biochemistry, pharmacology, and in vivo function of arginases.Pharmacological reviews · 2025Review
- Ocular Tissue-Specific Amino Acid Metabolism in Gyrate Atrophy.Advances in experimental medicine and biology · 2025Review
- Foxa deficiency restricts hepatitis B virus biosynthesis through epigenic silencing.Journal of virology · 2024Article
- Therapeutic Modulation of Arginase with nor-NOHA Alters Immune Responses in Experimental Mouse Models of Pulmonary Tuberculosis including in the Setting of Human Immunodeficiency Virus (HIV) Co-Infection.Tropical medicine and infectious disease · 2024Article
- Monocyte (THP-1) Response to Silver Nanoparticles Synthesized withNanomaterials (Basel, Switzerland) · 2024Article
- The role and control of arginine levels in arginase 1 deficiency.Journal of inherited metabolic disease · 2023Review
- Modelling urea cycle disorders using iPSCs.NPJ Regenerative medicine · 2022Review
- Stroke-Induced Neurological Dysfunction in Aged Mice Is Attenuated by Preconditioning with Young Sca-1+ Stem Cells.Stem cells (Dayton, Ohio) · 2022Article
- Article
- Collapse of the hepatic gene regulatory network in the absence of FoxA factors.Genes & development · 2020Article
- Topiramate Reverses Physiological and Behavioral Alterations by Postoperative Cognitive Dysfunction in Rat Model Through Inhibiting TNF Signaling Pathway.Neuromolecular medicine · 2020Article
- Myeloid Cell-Derived Arginase in Cancer Immune Response.Frontiers in immunology · 2020Review
- Genome editing technologies to treat rare liver diseases.Translational gastroenterology and hepatology · 2020Review
- InducibleInternational journal of molecular sciences · 2019Article
- Human hepatocyte transplantation corrects the inherited metabolic liver disorder arginase deficiency in mice.Molecular genetics and metabolism · 2018Article
- Transplantation of Gene-Edited Hepatocyte-like Cells Modestly Improves Survival of Arginase-1-Deficient Mice.Molecular therapy. Nucleic acids · 2018Article
- Critical role for arginase 2 in obesity-associated pancreatic cancer.Nature communications · 2017Article
- Proof-of-Concept Gene Editing for the Murine Model of Inducible Arginase-1 Deficiency.Scientific reports · 2017Article
- Liver-specific knockout of arginase-1 leads to a profound phenotype similar to inducible whole body arginase-1 deficiency.Molecular genetics and metabolism reports · 2016Article
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Arginase deficiency is a rare autosomal recessive disorder resulting from a loss of the liver arginase isoform, arginase 1 (ARG1), which is the final step in the urea cycle for detoxifying ammonia. ARG1 deficiency leads to hyperargininemia, characterized by progressive neurological impairment, persistent growth retardation and infrequent episodes of hyperammonemia. Using the Cre/loxP-directed conditional gene knockout system, we generated an inducible Arg1-deficient mouse model by crossing "floxed" Arg1 mice with CreER(T2) mice. The resulting mice (Arg-Cre) die about two weeks after tamoxifen administration regardless of the starting age of inducing the knockout. These treated mice were nearly devoid of Arg1 mRNA, protein and liver arginase activity, and exhibited symptoms of hyperammonemia. Plasma amino acid analysis revealed pronounced hyperargininemia and significant alterations in amino acid and guanidino compound metabolism, including increased citrulline and guanidinoacetic acid. Despite no alteration in ornithine levels, concentrations of other amino acids such as proline and the branched-chain amino acids were reduced. In summary, we have generated and characterized an inducible Arg1-deficient mouse model exhibiting several pathologic manifestations of hyperargininemia. This model should prove useful for exploring potential treatment options of ARG1 deficiency.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.