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

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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Bibliographic Details
Main Authors: Caporali, Paola, Bruno, Francesco, Palladino, Giampiero, Dragotto, Jessica, Petrosini, Laura, Mangia, Franco, Erickson, Robert P., Canterini, Sonia, Fiorenza, Maria Teresa
Other Authors: Univ Arizona, Dept Pediat
Language:en
Published: BIOMED CENTRAL LTD 2016
Subjects:
Lysosomal storage disorders
Cholesterol
Motor behavior
Cerebellar cortex development
2-hydroxypropyl-beta-cyclodextrin
Dysmyelination
Online Access:http://hdl.handle.net/10150/621982
http://arizona.openrepository.com/arizona/handle/10150/621982

Internet

http://hdl.handle.net/10150/621982
http://arizona.openrepository.com/arizona/handle/10150/621982

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