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Phenotypes Associated with This Genotype
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Iduatm1Clk mutation (2 available); any Idua mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
• only 21% of males and 40% of females live past 12 months of age
• the median survival age for males is 32 weeks and for females 48 weeks
• the rapid loss of male mice starts around 7 months of age and for females at 9 months of age

• body weight of mice becomes significantly greater than controls at 11 weeks of age for females and 14 weeks of age for males
• mice remain heavier for at 34 weeks of age

• 8 month old males bury fewer marblesthan controls, which is suggestive of increased anxiety
• control mice 8 months of age spent 66% of the time exploring a novel object whereas the mutant mice only spent 54% of the time exploring the novel object
• mice have less habituation to an open field as determined by less decrease in locomotor activity on the third exposure compared to the first
• 4-month old mice take longer than controls to reach a hidden platform in a morris water maze
• differences were not observed at 2, 6, and 8 months of age
• mice at 4 or 8 months of age swim further away from target than controls both 1 day and 7 days after last learning session in a morris water maze
• 61.0% of mutant mice do no react to an acoustic startle compared to 9.7% of control mice that do not react
• mice have decreased horizontal activity when placed into a new environment

• urinary secretion of glycosaminoglycans is 4- to 20-fold higher than controls regardless of age

renal/urinary system
• urinary secretion of glycosaminoglycans is 4- to 20-fold higher than controls regardless of age

Mouse Models of Human Disease
OMIM ID Ref(s)
Hurler Syndrome 607014 J:130215

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
MGI 6.01
The Jackson Laboratory