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Phenotypes Associated with This Genotype
Genotype
MGI:3580456
Allelic
Composition
Prkdcscid/Prkdcscid
Iduatm1Clk/Iduatm1Clk
Genetic
Background
NOD.129(B6)-Prkdcscid Iduatm1Clk/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Iduatm1Clk mutation (2 available); any Idua mutation (9 available)
Prkdcscid mutation (76 available); any Prkdc mutation (204 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
reproductive system
• matings between homozygote females and heterozygote males are unsuccessful

behavior/neurological
• mice trained on a rotating rod have decreased times of latency in subsequent tests compared to controls
• males have more deficient motor learning than females
• for females, there was some improvement in times of latency at all speeds though never to the degree seen for controls
• training failed to improve time of latency for males at the highest speed tested
• mice have decreased times of latency in rotarod tests
• males have worse impairment than females
• mothers often cannibalize their offspring

craniofacial
• mice have thickening of the zygomatic bone
• mice have a short snout that is more evident in females
• mice have broad head that is more evident in females
• mice have small ears that is more evident in females

hearing/vestibular/ear
• mice have small ears that is more evident in females

homeostasis/metabolism
• tissue levels of glycosaminoglycans are extremely high throughout tissues with highest levels in the liver followed by kidney, lung, spleen, heart and brain
• urinary excretion of glycosaminoglycans is 6.5-fold higher than in controls

nervous system
• ganglioside accumulations are observed
• high ganglioside accumulations are observed
• high ganglioside accumulations are observed
• ganglioside accumulations are observed
• ganglioside accumulations are observed
• ganglioside accumulations are observed

renal/urinary system
• urinary excretion of glycosaminoglycans is 6.5-fold higher than in controls

skeleton
• mice have thickening of the zygomatic bone

vision/eye
• mice have a short snout that is more evident in females

integument
• mice have a rough coat

growth/size/body
• mice have a short snout that is more evident in females
• mice have broad head that is more evident in females
• mice have small ears that is more evident in females

Mouse Models of Human Disease
OMIM ID Ref(s)
Hurler Syndrome 607014 J:139626


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
09/20/2016
MGI 6.05
The Jackson Laboratory