Developmental delay in motor skill acquisition in Niemann-Pick C1 mice reveals abnormal cerebellar morphogenesis

Niemann-Pick type C1 (NPC1) disease is a lysosomal storage disorder caused by defective intracellular trafficking of exogenous cholesterol. Purkinje cell (PC) degeneration is the main sign of cerebellar dysfunction in both NPC1 patients and animal models. It has been recently shown that a significan...

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Detalles Bibliográficos
Autores principales: Caporali, Paola, Bruno, Francesco, Palladino, Giampiero, Dragotto, Jessica, Petrosini, Laura, Mangia, Franco, Erickson, Robert P., Canterini, Sonia, Fiorenza, Maria Teresa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2016
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5009663/
https://www.ncbi.nlm.nih.gov/pubmed/27586038
http://dx.doi.org/10.1186/s40478-016-0370-z

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5009663/
https://www.ncbi.nlm.nih.gov/pubmed/27586038
http://dx.doi.org/10.1186/s40478-016-0370-z

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