NPC1 deficiency impairs cerebellar postnatal development of microglia and climbing fiber refinement in a mouse model of Niemann-Pick disease type C.

Authors

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 Carone, Rowan UniversityFollow
Ileana Soto Reyes, Rowan UniversityFollow

Document Type

Article

Version Deposited

Published Version

Publication Date

8-3-2020

Publication Title

Development (Cambridge, England)

DOI

10.1242/dev.189019

Abstract

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

Comments

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution and reproduction in any medium provided that the original work is properly attributed.

Recommended Citation

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

Creative Commons License

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