Phenotypes Associated with This Genotype

Genotype
MGI:5525128

• Allelic Composition: Tg(Prnp-ITM2B*)1Ruvi/Tg(Prnp-ITM2B*)1Ruvi
• Genetic Background: involves: C3HeB/FeJ * C57BL/6

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

behavior/neurological

abnormal grooming behavior ( J:197198 )

• abnormal grooming behavior starting around 9 months of age as evident by dull rough coats

limb grasping ( J:197198 )

• at 12 months of age, 77% of mutants exhibit cupping of the hind paws and bilaterally pulling of the hind paws toward the abdomen when suspended by the tail compared to 33% of wild-type mice

hunched posture ( J:197198 )

• by 12 months of age, some mutants show a hunched back or arched posture when sitting and walking

abnormal gait ( J:197198 )

• 12 month old mutants exhibit an usual gait in which the mouse holds its body near the walking surface and takes wide shortened steps

short stride length ( J:197198 )

growth/size/body

weight loss ( J:197198 )

• weight loss by 6 months of age

nervous system

abnormal astrocyte morphology ( J:197198 )

• increase in expression of glial fibrillary acidic protein, indicating activation of astrocytes

abnormal neurite morphology ( J:197198 )

• presence of clusters of swollen neurites at the periphery of Danish amyloid plaques

• these dystrophic neurites are made up of large and rounded processes

abnormal synapse morphology ( J:197198 )

• with age, synaptophysin levels decrease indicating synaptic degeneration

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
cerebral amyloid angiopathy		DOID:9246		J:197198