Please use this identifier to cite or link to this item: http://repositorio.unicamp.br/jspui/handle/REPOSIP/326630
Type: Artigo
Title: Lithium Carbonate And Coenzyme Q10 Reduce Cell Death In A Cell Model Of Machado-joseph Disease
Author: Lopes-Ramos
C. M.; Pereira
T. C.; Dogini
D. B.; Gilioli
R.; Lopes-Cendes
I.
Abstract: Machado-Joseph disease (MJD) or spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant neurodegenerative disorder caused by expansion of the polyglutamine domain of the ataxin-3 (ATX3) protein. MJD/SCA3 is the most frequent autosomal dominant ataxia in many countries. The mechanism underlying MJD/SCA3 is thought to be mainly related to protein misfolding and aggregation leading to neuronal dysfunction followed by cell death. Currently, there are no effective treatments for patients with MJD/SCA3. Here, we report on the potential use of lithium carbonate and coenzyme Q10 to reduce cell death caused by the expanded ATX3 in cell culture. Cell viability and apoptosis were evaluated by MTT assay and by flow cytometry after staining with annexin V-FITC/propidium iodide. Treatment with lithium carbonate and coenzyme Q10 led to a significant increase in viability of cells expressing expanded ATX3 (Q84). In addition, we found that the increase in cell viability resulted from a significant reduction in the proportion of apoptotic cells. Furthermore, there was a significant change in the expanded ATX3 monomer/aggregate ratio after lithium carbonate and coenzyme Q10 treatment, with an increase in the monomer fraction and decrease in aggregates. The safety and tolerance of both drugs are well established; thus, our results indicate that lithium carbonate and coenzyme Q10 are good candidates for further in vivo therapeutic trials.
Subject: Machado-joseph Disease
Spinocerebellar Ataxia Type 3
Lithium Carbonate
Coenzyme Q10
Drug Treatment
Editor: Assoc Bras Divulg Cientifica
São Paulo
Rights: aberto
Identifier DOI: 10.1590/1414-431X20165805
Address: http://www.scielo.br/scielo.php?pid=S0100-879X2016001200601&script=sci_arttext
Date Issue: 2016
Appears in Collections:Unicamp - Artigos e Outros Documentos

Files in This Item:
File SizeFormat 
000389235000003.pdf722.94 kBAdobe PDFView/Open


Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.