Ambroxol-induced rescue of defective glucocerebrosidase is associated with increased LIMP-2 and saposin C levels in GBA1 mutant Parkinson's disease cells
详细信息 查看全文
- 作者：Giulia Ambrosi ; Cristina Ghezzi ; Roberta Zangaglia ; Giovanna Levandis ; Claudio Pacchetti ; Fabio Blandini ; fabio.blandini@mondino.it" class="auth_mail" title="E-mail the corresponding author
- 关键词：PD ; Parkinson's disease ; GD ; Gaucher disease ; GCase ; glucocerebrosidase ; Sap C ; saposin C ; PSAP ; prosaposin ; LIMP-2 ; lysosomal integral membrane 2 ; ER ; endoplasmic reticulum ; TFEB ; transcription factor EB ; GSK3 ; glycogen synthase kinase 3 ; UPS ; ubiquitin proteasome system
- 刊名：Neurobiology of Disease
- 出版年：2015
- 出版时间：October 2015
- 年：2015
- 卷：82
- 期：Complete
- 页码：235-242
- 全文大小：1102 K
文摘
- listitem" id="list_l0005">
- •
Glucocerebrosidase is defective in PD fibroblasts carrying GBA1 mutations.
- •
Ambroxol increases glucocerebrosidase activity and protein levels in all cells.
- •
Ambroxol increases saposin C protein levels and cathepsin D activity in all cells.
- •
Ambroxol increases LIMP-2 levels only in GBA1-mutant PD cells.