Breaches of the pial basement membrane are associated with defective dentate gyrus development in mouse models of congenital muscular dystrophies |
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Authors: | Li Jing Yu Miao Feng Gang Hu Huaiyu Li Xiaofeng |
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Institution: | a Department of Neuroscience and Physiology, SUNY Upstate Medical University, Syracuse, NY 13210, USA b Department of Neurology, The Second Affiliated Hospital of Chongqing Medical University, Chongqing, PR China |
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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 Largemyd 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 Largemyd 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. |
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Keywords: | Congenital muscular dystrophies Dentate gyrus Brain development Basement membrane |
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