Phenotypes associated with this allele

• Allele Symbol: Prelp^tm1.1Sohm
• Allele Name: targeted mutation 1.1, Shin-Ichi Ohnuma
• Allele ID: MGI:8249485
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Prelp^tm1.1Sohm/Prelp^tm1.1Sohm	B6(Cg)-Prelp^tm1.1Sohm	MGI:8252943

Genotype
MGI:8252943

hm1

• Allelic Composition: Prelp^tm1.1Sohm/Prelp^tm1.1Sohm
• Genetic Background: B6(Cg)-Prelp^tm1.1Sohm

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

nervous system

abnormal brain vasculature morphology ( J:342336 )

• cerebellar vasculature exhibits weakened cell-cell adhesion with a significant reduction in the immunostaining intensity of VE-cadherin (an adherens junction marker) and two tight junction markers, claudin-5 and ZO-1

impaired blood-brain barrier function ( J:342336 )

• at 1 year of age, the integrity of the blood-brain barrier (BBB) is disrupted, resulting in IgG and dextran leakage in the cerebellum and cortex

• % of IgG detected outside of the vasculature is significantly higher than in control brains and disruption of BBB is more apparent in the cerebellum than in the cortex

• analysis of neurovascular unit (NVU) components shows that the immunostaining intensity of laminin is lost, esp. at sites with intense IgG leakage, while perlecan staining is significantly reduced

• however, brain water content is not significantly altered

abnormal astrocyte morphology ( J:342336 )

• double staining with AQP4 (a water channel found on astrocytic end-feet) and IgG in the cerebellum reveals a significant decrease in AQP4 signal in astrocyte perivascular end-feet

• however, astrocyte number, morphology, and staining intensity remain normal

microgliosis ( J:342336 )

• mice show a significant increase in the number of Iba-1 positive microglia in the cerebellum

increased microglial cell activation ( J:342336 )

• mice show a significant decrease in microglial branch length per microglial density in the cerebellum, indicating an increased microglial response

CNS inflammation ( J:342336 )

• expression profiling analysis of meninges and immunohistochemical analysis of microglia indicate the presence of neuroinflammation in the brain

cardiovascular system

abnormal brain vasculature morphology ( J:342336 )

• cerebellar vasculature exhibits weakened cell-cell adhesion with a significant reduction in the immunostaining intensity of VE-cadherin (an adherens junction marker) and two tight junction markers, claudin-5 and ZO-1

abnormal pericyte morphology ( J:342336 )

• in the brain, the point of association between endothelial cells with pericytes is reduced, suggesting pericyte detachment from capillaries

impaired blood-brain barrier function ( J:342336 )

• at 1 year of age, the integrity of the blood-brain barrier (BBB) is disrupted, resulting in IgG and dextran leakage in the cerebellum and cortex

• % of IgG detected outside of the vasculature is significantly higher than in control brains and disruption of BBB is more apparent in the cerebellum than in the cortex

• analysis of neurovascular unit (NVU) components shows that the immunostaining intensity of laminin is lost, esp. at sites with intense IgG leakage, while perlecan staining is significantly reduced

• however, brain water content is not significantly altered

cellular

abnormal astrocyte morphology ( J:342336 )

• double staining with AQP4 (a water channel found on astrocytic end-feet) and IgG in the cerebellum reveals a significant decrease in AQP4 signal in astrocyte perivascular end-feet

• however, astrocyte number, morphology, and staining intensity remain normal

microgliosis ( J:342336 )

• mice show a significant increase in the number of Iba-1 positive microglia in the cerebellum

decreased cell adhesion ( J:342336 )

• mice exhibit weakened cell-cell contacts between vascular smooth muscle cells

• cerebellar vasculature shows a significant reduction in the immunostaining intensity of adhesion junction proteins such as VE-cadherin, claudin-5, and ZO-1

increased microglial cell activation ( J:342336 )

• mice show a significant decrease in microglial branch length per microglial density in the cerebellum, indicating an increased microglial response

abnormal basement membrane morphology ( J:342336 )

• analysis of basement membrane components shows that the immunostaining intensity of laminin is lost, esp. at sites with intense IgG leakage, while perlecan staining is significantly reduced

• however, no significant reduction of collagen IV staining is observed, suggesting that collagen IV expression is maintained independent of IgG leakage

immune system

microgliosis ( J:342336 )

• mice show a significant increase in the number of Iba-1 positive microglia in the cerebellum

increased microglial cell activation ( J:342336 )

• mice show a significant decrease in microglial branch length per microglial density in the cerebellum, indicating an increased microglial response

CNS inflammation ( J:342336 )

• expression profiling analysis of meninges and immunohistochemical analysis of microglia indicate the presence of neuroinflammation in the brain

hematopoietic system

microgliosis ( J:342336 )

• mice show a significant increase in the number of Iba-1 positive microglia in the cerebellum

increased microglial cell activation ( J:342336 )

• mice show a significant decrease in microglial branch length per microglial density in the cerebellum, indicating an increased microglial response