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Phenotypes Associated with This Genotype
Genotype
MGI:4415196
Allelic
Composition
Iduatm1.1Kmke/Iduatm1.1Kmke
Genetic
Background
B6.129-Iduatm1.1Kmke
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Iduatm1.1Kmke mutation (1 available); any Idua mutation (9 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mean lifespan is 69 weeks

skeleton
• the zygomatic arch is slightly thickened at 15 weeks of age and more prominently so at 35 weeks of age compared to in wild-type mice
• femur width is increased 1.13-fold at 15 weeks and 1.37-fold at 35 weeks compared to in wild-type mice
• 15% at 5 weeks but no longer detectable at 35 weeks of age
• 25% at 35 weeks in the femur

cellular
• foamy cells distended by glycosamineglycans (GAGs) accumulation are observed in the liver, spleen, myocardium, aorta, kidney, and lung unlike in wild-type mice
• foamy cell infiltration worsens with age
• at 30 weeks, cytoplasmic inclusions consisting of lysosomal storage material are found in Purkinje cells of the cerebellum and in neurons of the medulla unlike in wild-type mice

adipose tissue

renal/urinary system
• the levels of glycosaminoglycans (GAGs) in the urine is increased 2.5-fold, 3.6-fold, and 5.6-fold at 5, 10, and 30 weeks, respectively, compared to in wild-type mice

craniofacial
• the zygomatic arch is slightly thickened at 15 weeks of age and more prominently so at 35 weeks of age compared to in wild-type mice
• the snout is slightly broadened at 10 to 15 week of age and more prominently broadened at 35 weeks of age compared to in wild-type mice

homeostasis/metabolism
• the levels of glycosaminoglycans (GAGs) in the urine is increased 2.5-fold, 3.6-fold, and 5.6-fold at 5, 10, and 30 weeks, respectively, compared to in wild-type mice

limbs/digits/tail
• femur width is increased 1.13-fold at 15 weeks and 1.37-fold at 35 weeks compared to in wild-type mice
• 15% at 5 weeks but no longer detectable at 35 weeks of age

growth/size/body
• the snout is slightly broadened at 10 to 15 week of age and more prominently broadened at 35 weeks of age compared to in wild-type mice

Mouse Models of Human Disease
OMIM ID Ref(s)
Hurler Syndrome 607014 J:155619


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
06/22/2016
MGI 6.04
The Jackson Laboratory