Phenotypes Associated with This Genotype

Genotype
MGI:3839661

• Allelic Composition: Idua^tm1Clk/Idua^tm1Clk
• Genetic Background: B6.129-Idua^tm1Clk/J

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

decreased survivor rate ( J:130215 )

• 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

growth/size/body

increased body weight ( J:130215 )

• 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

behavior/neurological

abnormal learning/memory/conditioning ( J:130215 )

• mice have less habituation to an open field as determined by less decrease in locomotor activity on the third exposure compared to the first

abnormal spatial learning ( J:130215 )

• 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

abnormal long-term spatial reference memory ( J:130215 )

• 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

increased anxiety-related response ( J:130215 )

• 8 month old males bury fewer marblesthan controls, which is suggestive of increased anxiety

abnormal response to novel object ( J:130215 )

• 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

decreased startle reflex ( J:130215 )

• 61.0% of mutant mice do no react to an acoustic startle compared to 9.7% of control mice that do not react

decreased locomotor activity ( J:130215 )

• mice have decreased horizontal activity when placed into a new environment

homeostasis/metabolism

increased urine glycosaminoglycan level ( J:130215 )

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

renal/urinary system

increased urine glycosaminoglycan level ( J:130215 )

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

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
mucopolysaccharidosis I		DOID:12802		J:130215