Cerebellar corticogenesis in the lysosomal acid phosphatase (acp2) mutant mice: purkinje cell migration disorder
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Date
2017
Authors
Ashtari, Niloufar
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Abstract
In a mutant mouse called nax as the result of mutation in Lysosomal Acid phosphatase (Acp2), layers of the cerebellar cortex are impaired and monolayer Purkinje cells (Pcs) turn to multi-layered Pcs that ectopically invade the molecular layer. We investigated reelin-Dab1 signaling as an important pathway for Pcs migration and monolayer formation in cerebellum. ERK1/2 is a member of mitogen activated kinases family and suggested to be a downstream of reelin signaling. We hypothesize that the establishment of mono-layered Pcs rely on reelin through ERK1/2 pathway. Acp2 mutant mice were used for this study and molecular expression and distribution were assessed by immunohistochemistry, RT-PCR, western blotting, and cell culture. Results suggest that reelin may modulate the ERK1/2 expression, thus lower expression of reelin and higher phosphorylation of Dab1 leads to over expression of the ERK1/2 that causes the Pcs to over migrate and form multilayer in nax cerebellar cortex.
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Keywords
Cerebellum, Purkinje cell, Migration, Development, Acp2