Phenotypes Associated with This Genotype

Genotype
MGI:4365903

• Allelic Composition: Tg(tetO/CMV-Prnp*,-lacZ)33Mjy/Tg(tetO/CMV-Prnp*,-lacZ)33Mjy; Tg(Camk2a-rtTA)1237Kndl/0
• Genetic Background: involves: FVB/N

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

growth/size/body

decreased body size ( J:153494 )

• smaller and noticeable from 14 days after birth

• no obvious difference in food intake

• this and all subsequent phenotypes are reported when transgene expression is never suppressed

postnatal growth retardation ( J:153494 )

• progresses most rapidly from 14 days to 4 weeks after birth

• the body size of homozygous mice remains smaller

behavior/neurological

limb grasping ( J:153494 )

circling ( J:153494 )

• stereotypical circling

nervous system

astrocytosis ( J:153494 )

• in cortical and hippocampal regions

abnormal hippocampus morphology ( J:153494 )

• more condensed nuclei are observed in hippocampal neurons

• reduced neurofilament staining in the hippocampal regions

abnormal cerebral cortex morphology ( J:153494 )

• more condensed nuclei are observed in cortical neurons

• reduced neurofilament staining in the cortical regions

thin cerebral cortex ( J:153494 )

• thinner cortex, more severe

neurodegeneration ( J:153494 )

• more condensed nuclei are observed in cortical and hippocampal neurons

• reduced neurofilament staining in the cortical and hippocampal regions

cellular

abnormal cell morphology ( J:153494 )

• majority of cyPrP was aggregated in aged (190 days) homozygous mice brain

• a significant portion of cyPrP was soluble in 24-day-old homozygous transgenic mice

astrocytosis ( J:153494 )

• in cortical and hippocampal regions

abnormal plasma membrane morphology ( J:153494 )

• a portion of cyPrP is membrane bound

• cyPrP is associated with the hydrophobic lipid core of membranes

• cyPrP forms ordered amyloid oligomer