title: Breaches of the pial basement membrane are associated with defective dentate gyrus development in mouse models of congenital muscular dystrophies.
abstract: A subset of congenital muscular dystrophies (CMDs) has central nervous system manifestations. There are good mouse models for these CMDs that include POMGnT1 knockout, POMT2 knockout and Large(myd) mice with all exhibiting defects in dentate gyrus. It is not known how the abnormal dentate gyrus is formed during the development. In this study, we conducted a detailed morphological examination of the dentate gyrus in adult and newborn POMGnT1 knockout, POMT2 knockout, and Large(myd) mice by immunofluorescence staining and electron microscopic analyses. We observed that the pial basement membrane overlying the dentate gyrus was disrupted and there was ectopia of granule cell precursors through the breached pial basement membrane. Besides these, the knockout dentate gyrus exhibited reactive gliosis in these mouse models. Thus, breaches in the pial basement membrane are associated with defective dentate gyrus development in mouse models of congenital muscular dystrophies.
authors.affiliation: Department of Neuroscience and Physiology, SUNY Upstate Medical University, Syracuse, NY 13210, USA.
authors.forename: Jing
authors.initials: J
authors.lastname: Li
authors.forename: Miao
authors.initials: M
authors.lastname: Yu
authors.forename: Gang
authors.initials: G
authors.lastname: Feng
authors.forename: Huaiyu
authors.initials: H
authors.lastname: Hu
authors.forename: Xiaofeng
authors.initials: X
authors.lastname: Li
mesh_terms: D000818:Animals; D001485:Basement Membrane / Q000002:abnormalities* / Q000378:metabolism / Q000648:ultrastructure; D018891:Dentate Gyrus / Q000473:pathology*; D004195:Disease Models, Animal; D005786:Gene Expression Regulation / Q000235:genetics; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D005911:Gliosis / Q000235:genetics / Q000473:pathology; D018398:Homeodomain Proteins / Q000378:metabolism; D008364:Mannosyltransferases / Q000235:genetics / Q000378:metabolism; D051379:Mice; D018345:Mice, Knockout; D046529:Microscopy, Electron, Transmission; D009136:Muscular Dystrophies / Q000235:genetics* / Q000473:pathology*; D017351:N-Acetylglucosaminyltransferases / Q000172:deficiency / Q000235:genetics; D010841:Pia Mater / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D025521:Tumor Suppressor Proteins / Q000378:metabolism; D000098551:Prospero-Related Homeobox 1 Protein
chemical_list: D005904:Glial Fibrillary Acidic Protein; D018398:Homeodomain Proteins; D025521:Tumor Suppressor Proteins; D000098551:Prospero-Related Homeobox 1 Protein; C502747:Large1 protein, mouse; D008364:Mannosyltransferases; D017351:N-Acetylglucosaminyltransferases; C443106:protein O-mannose beta-1,2-N-acetylglucosaminyltransferase; C082696:protein O-mannosyltransferase