Development (Cambridge, England)
Little is known about the effects of NPC1 deficiency in brain development and whether these effects contribute to neurodegeneration in Niemann-Pick disease type C (NPC). Degeneration of cerebellar Purkinje cells occurs at an earlier stage and to a greater extent in NPC; therefore, we analyzed the effect of NPC1 deficiency on microglia and on climbing fiber synaptic refinement during cerebellar postnatal development using the
Bridget R. Boyle, Sierra E. Melli, Ruth S. Altreche, Zachary M. Padron, Fawad A. K. Yousufzai, Sarah Kim, Mariella D. Vasquez, Dawn M. Carone, Benjamin R. Carone, & Ileana Soto. (2020) NPC1 deficiency impairs cerebellar postnatal development of microglia and climbing fiber refinement in a mouse model of Niemann–Pick disease type C. Development 2020, 147: dev189019
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