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Heat shock protein amplification improves cerebellar myelination in the Npc1(nih) mouse model

BACKGROUND: Niemann-Pick disease type C (NPC) is a rare prematurely fatal lysosomal lipid storage disease with limited therapeutic options. The prominent neuropathological hallmarks include hypomyelination and cerebellar atrophy. We previously demonstrated the efficacy of recombinant human heat shoc...

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Detalles Bibliográficos
Autores principales: Gray, James, Fernández-Suárez, María E., Falah, Maysa, Smith, David, Smith, Claire, Kaya, Ecem, Palmer, Ashley M., Fog, Cathrine K., Kirkegaard, Thomas, Platt, Frances M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9713282/
https://www.ncbi.nlm.nih.gov/pubmed/36455410
http://dx.doi.org/10.1016/j.ebiom.2022.104374