Mouse Genome Informatics
Not Specified
phenotype observed in females
phenotype observed in males
N normal phenotype
• die at postnatal day 10-14

• total kidney arginase activity levels normal; ARG2 levels are thought to be induced two-fold in these mice
• elevated plasma arginine levels: four fold greater than the level of wild-type
• reduced plasma proline levels: one third of the level of wild-type
• reduced plasma ornithine levels: one half of the level of wild-type
• elevated plasma glutamic acid, citrulline and histidine levels: about 1.5 fold greater than the wild-type level
• reduced plasma valine, isoleucine and leucine levels: 0.4 to 0.5 of the level of wild-type
• plasma ammonia levels are increased 10 fold in mice exhibiting metabolic crisis

liver/biliary system
• nuclei and cytoplasm enlarged two to three fold in most cells; cytoplasmic inclusions also noted as well as other abnormalities; abnormal nuclei

nervous system
• normal brain morphology; no astrocyte defects noted

Mouse Models of Human Disease
Argininemia 207800 J:83263