Int J Mol Sci 2021 Mar 15;22(6). Epub 2021 Mar 15.
Department of Human Anatomy and Cell Science, The Children's Hospital Research Institute of Manitoba (CHRIM), Rady Faculty of Health Sciences, Max Rady College of Medicine, University of Manitoba, Winnipeg, MB R3E 0W2, Canada.
Lysosomal acid phosphatase 2 () mutant mice (naked-ataxia, ) have a severe cerebellar cortex defect with a striking reduction in the number of granule cells. Using a combination of in vivo and in vitro immunohistochemistry, Western blotting, BrdU assays, and RT-qPCR, we show downregulation of MYCN and dysregulation of the SHH signaling pathway in the cerebellum. MYCN protein expression is significantly reduced at P10, but not at the peak of proliferation at around P6 when the number of granule cells is strikingly reduced in the cerebellum. Read More