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Phenotypes Associated with This Genotype
Genotype
MGI:6303809
Allelic
Composition
Fkrptm1Scbr/?
Genetic
Background
Not Specified
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fkrptm1Scbr mutation (1 available); any Fkrp mutation (13 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• disruption of the radial glial scaffold, with radial glial processes often haphazardly arranged and complete disruption of the glia limitans
• no marginal zone is seen
• majority of Cajal-Retzius cells are located around the anterior cerebral artery and scattered Cajal-Retzius cells are seen within the disorganized cortical plate unlike in wild-type mice where they are present multifocally within the superficial molecular layer
• decrease in the number of Cajal-Retzius cells in the rostral cortex at the level of the corpus callosum and a concurrent increase in the number of Cajal-Retzius cells at the level of the hippocampus, suggesting a failure of tangential migration of these cells
• the cortical plate is not apparent
• 2 of 5 mice exhibit subtle cerebellar lesions characterized by disruption of the external granule cell layer
• in 3 of 5 mice
• 3 of 5 mice exhibit dilation of the lateral ventricles indicative of hydrocephalus
• abnormalities in the tectum but to a lesser extent than in the cortex
• the inferior and superior colliculi are disrupted, with substantial defects in the pia where large numbers of cells form a prominent subarachnoid layer
• the inferior and superior colliculi are disrupted, with substantial defects in the pia where large numbers of cells form a prominent subarachnoid layer
• 2 of 5 mice show disrupted dentate gyrus of the hippocampus
• the rostral cortex is disrupted at P0
• rostral cortex shows a complete absence of laminar organization, with no obvious structure to the cortical plate, and fusion of the interhemispheric fissure
• at the hippocampus level, the lateral aspects of the cortex are extensively disrupted while the cortical plate is established and there is incomplete formation of the interhemispheric fissure with interdigitation of neurons from opposing cortical plates
• at the level of the corpus callosum, disorganization is more pronounced laterally
• mice exhibit a rostrocaudal gradient in the severity of brain lesions, with lesions most pronounced in the rostral cortex and progressively milder more caudally
• fusion of the cortical hemispheres with interdigitation of neurons from opposing cortical plates
• leptomeningeal architecture is disrupted, with little distinction between the superficial arachnoid mater, the subarachnoid space, and the pia matter
• the pia is not apparent
• large numbers of heterotopic neurons and glial cells are present superficial to the glia limitans, expanding the subarachnoid space

cellular
• disruption of the radial glial scaffold, with radial glial processes often haphazardly arranged and complete disruption of the glia limitans


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
07/09/2019
MGI 6.14
The Jackson Laboratory