Mouse Genome Informatics
cx
    Prkdcscid/Prkdcscid
Iduatm1Clk/Iduatm1Clk

NOD.129(B6)-Prkdcscid Iduatm1Clk/J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
reproductive system
• matings between homozygote females and heterozygote males are unsuccessful (J:139626)

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 (J:139626)

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 IDRef(s)
Hurler Syndrome 607014 J:139626