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- W2092709351 abstract "The meninges produce essential signaling molecules and major protein components of the pial basement membrane during normal brain development. Disruptions in the pial basement membrane underlie neural ectopia seen in those congenital muscular dystrophies (CMDs) caused by mutations in genes involved in O-mannosyl glycosylation. In mammals, biosynthesis of O-mannosyl glycans is initiated by a complex of mutually indispensable protein O-mannosyltransferases 1 and 2 (POMT1 and 2). To study the roles of O-mannosylation in brain development we generated a conditional allele of POMT2. POMT2 nulllizygosity resulted in embryonic lethality because of a defective Reichert's membrane. Brain-specific deletion of POMT2 resulted in hypoglycosylation of α-dystroglycan (DG) and abolished laminin binding activity. The effect of POMT2 deletion on brain development was dependent on timing, as earlier deletion resulted in more severe phenotypes. Multiple brain malformations including overmigration of neocortical neurons and migration failure of granule cells in the cerebellum were observed. Immunofluorescence staining and transmission electron microscopy revealed that these migration defects were closely associated with disruptions in the pial basement membrane. Interestingly, POMT2 deletion in the meninges (and blood vessels) did not disrupt the development of the neocortex. Thus, normal brain development requires protein O-mannosylation activity in neural tissue but not the meninges. These results suggest that gene therapy should be directed to the neural tissue instead of the meninges." @default.
- W2092709351 created "2016-06-24" @default.
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- W2092709351 creator A5075173381 @default.
- W2092709351 date "2011-03-30" @default.
- W2092709351 modified "2023-10-12" @default.
- W2092709351 title "Conditional knockout of protein O-mannosyltransferase 2 reveals tissue-specific roles of O-mannosyl glycosylation in brain development" @default.
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- W2092709351 doi "https://doi.org/10.1002/cne.22572" @default.
- W2092709351 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3634366" @default.
- W2092709351 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/21452199" @default.
- W2092709351 hasPublicationYear "2011" @default.
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