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

James Gray,María E Fernández-Suárez,Maysa Falah,David Smith, Claire Smith,Ecem Kaya,Ashley M Palmer,Cathrine K Fog,Thomas Kirkegaard,Frances M Platt

EBioMedicine（2022）

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摘要

Funding for this study was provided by Orphazyme A/S (Copenhagen, Denmark) and a Pathfinder Award from The Wellcome Trust.

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关键词

BALB/cNctr-Npc1(m1N)/J,Fyn kinase,HSP70,NPC,Niemann-Pick disease type C,Npc1(nih),arimoclomol,bimoclomol,cholesterol,glycosphingolipids,heat shock proteins,lysosomal storage diseases,mice,myelination,neurodegeneration

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