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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


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Creative Commons Attribution 4.0 License
This work is licensed under a Creative Commons Attribution 4.0 License.

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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