Aqueous extract of Glycyrrhiza inflata inhibits aggregation by upregulating PPARGC1A and NFE2L2–ARE pathways in cell models of spinocerebellar ataxia 3

2014 ◽  
Vol 71 ◽  
pp. 339-350 ◽  
Author(s):  
Chiung-Mei Chen ◽  
Yu-Ting Weng ◽  
Wan-Ling Chen ◽  
Te-Hsien Lin ◽  
Chih-Ying Chao ◽  
...  
Keyword(s):  
Spinocerebellar Ataxia ◽  
Aqueous Extract ◽  
Cell Models ◽  
Glycyrrhiza Inflata ◽  
Spinocerebellar Ataxia 3

scholarly journals Aqueous Extract ofPaeonia lactifloraand Paeoniflorin as Aggregation Reducers Targeting Chaperones in Cell Models of Spinocerebellar Ataxia 3

2013 ◽  
Vol 2013 ◽  
pp. 1-11 ◽  
Author(s):  
Kuo-Hsuan Chang ◽  
Wan-Ling Chen ◽  
Li-Ching Lee ◽  
Chih-Hsin Lin ◽  
Pin-Jui Kung ◽  
...  
Keyword(s):  
Spinocerebellar Ataxia ◽  
Aqueous Extract ◽  
Cag Repeats ◽  
Cell Models ◽  
Working Mechanism ◽  
Starting Point ◽  
Wide Range ◽  
Polyq Diseases ◽  
Spinocerebellar Ataxia 3 ◽  
Polyq Tract

Spinocerebellar ataxia (SCA) types 1, 2, 3, 6, 7, and 17 as well as Huntington’s disease are a group of neurodegenerative disorders caused by expanded CAG repeats encoding a long polyglutamine (polyQ) tract in the respective proteins. Evidence has shown that the accumulation of intranuclear and cytoplasmic misfolded polyQ proteins leads to apoptosis and cell death. Thus suppression of aggregate formation is expected to inhibit a wide range of downstream pathogenic events in polyQ diseases. In this study, we established a high-throughput aggregation screening system using 293 ATXN3/Q75-GFP cells and applied this system to test the aqueous extract ofPaeonia lactiflora(P. lactiflora) and its constituents. We found that the aggregation can be significantly prohibited byP. lactifloraand its active compound paeoniflorin. Meanwhile,P. lactifloraand paeoniflorin upregulated HSF1 and HSP70 chaperones in the same cell models. Both of them further reduced the aggregation in neuronal differentiated SH-SY5Y ATXN3/Q75-GFP cells. Our results demonstrate howP. lactifloraand paeoniflorin are likely to work on polyQ-aggregation reduction and provide insight into the possible working mechanism ofP. lactiflorain SCA3. We anticipate our paper to be a starting point for screening more potential herbs for the treatment of SCA3 and other polyQ diseases.

Novel Lactulose and Melibiose Targeting Autophagy to Reduce PolyQ Aggregation in Cell Models of Spinocerebellar Ataxia 3

2016 ◽  
Vol 15 (3) ◽  
pp. 351-359 ◽  
Author(s):  
Chih-Hsin Lin ◽  
Yih-Ru Wu ◽  
Jinn-Moon Yang ◽  
Wan-Ling Chen ◽  
Chih-Ying Chao ◽  
...  
Keyword(s):  
Spinocerebellar Ataxia ◽  
Cell Models ◽  
Spinocerebellar Ataxia 3

Unusual nocturnal motor restlessness in a patient with spinocerebellar ataxia 3

2005 ◽  
Vol 20 (7) ◽  
pp. 899-901 ◽  
Author(s):  
Imad Ghorayeb ◽  
Federica Provini ◽  
Bernard Bioulac ◽  
François Tison
Keyword(s):  
Spinocerebellar Ataxia ◽  
Motor Restlessness ◽  
Spinocerebellar Ataxia 3

Multiple system atrophy in a patient with the spinocerebellar ataxia 3 gene mutation

2007 ◽  
Vol 22 (2) ◽  
pp. 251-253 ◽  
Author(s):  
Melissa J. Nirenberg ◽  
Jenny Libien ◽  
Jean-Paul Vonsattel ◽  
Stanley Fahn
Keyword(s):  
Multiple System Atrophy ◽  
Gene Mutation ◽  
Spinocerebellar Ataxia ◽  
Spinocerebellar Ataxia 3

Spinocerebellar ataxia 3 and machado-joseph disease: Clinical, molecular, and neuropathological features

1996 ◽  
Vol 39 (4) ◽  
pp. 490-499 ◽  
Author(s):  
Alexandra Durr ◽  
Giovanni Stevanin ◽  
Geraldine Cancel ◽  
Charles Duyckaerts ◽  
Nacer Abbas ◽  
...  
Keyword(s):  
Spinocerebellar Ataxia ◽  
Machado Joseph Disease ◽  
Spinocerebellar Ataxia 3
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