Protective effect of encapsulated cells producing neurotrophic factor CNTF in a monkey model of Huntington's disease

Nature ◽  
1997 ◽  
Vol 386 (6623) ◽  
pp. 395-399 ◽  
Author(s):  
Dwaine F. Emerich ◽  
Shelley R. Winn ◽  
Philippe M. Hantraye ◽  
Marc Peschanski ◽  
Er-Yun Chen ◽  
...  
Keyword(s):  
Protective Effect ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neurotrophic Factor ◽  
Encapsulated Cells ◽  
Monkey Model

scholarly journals Cerebral dopamine neurotrophic factor (CDNF) protects against quinolinic acid-induced toxicity in in vitro and in vivo models of Huntington’s disease

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
P. Stepanova ◽  
V. Srinivasan ◽  
D. Lindholm ◽  
M. H. Voutilainen
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Quinolinic Acid ◽  
Neurotrophic Factor ◽  
Striatal Neurons ◽  
In Vivo Models ◽  
Cerebral Dopamine Neurotrophic Factor ◽  
Cerebral Dopamine

Abstract Huntington’s disease (HD) is a neurodegenerative disorder with a progressive loss of medium spiny neurons in the striatum and aggregation of mutant huntingtin in the striatal and cortical neurons. Currently, there are no rational therapies for the treatment of the disease. Cerebral dopamine neurotrophic factor (CDNF) is an endoplasmic reticulum (ER) located protein with neurotrophic factor (NTF) properties, protecting and restoring the function of dopaminergic neurons in animal models of PD more effectively than other NTFs. CDNF is currently in phase I–II clinical trials on PD patients. Here we have studied whether CDNF has beneficial effects on striatal neurons in in vitro and in vivo models of HD. CDNF was able to protect striatal neurons from quinolinic acid (QA)-induced cell death in vitro via increasing the IRE1α/XBP1 signalling pathway in the ER. A single intrastriatal CDNF injection protected against the deleterious effects of QA in a rat model of HD. CDNF improved motor coordination and decreased ataxia in QA-toxin treated rats, and stimulated the neurogenesis by increasing doublecortin (DCX)-positive and NeuN-positive cells in the striatum. These results show that CDNF positively affects striatal neuron viability reduced by QA and signifies CDNF as a promising drug candidate for the treatment of HD.

scholarly journals Brain-Derived Neurotrophic Factor in Patients with Huntington's Disease

PLoS ONE ◽  
2011 ◽  
Vol 6 (8) ◽  
pp. e22966 ◽  
Author(s):  
Chiara Zuccato ◽  
Manuela Marullo ◽  
Barbara Vitali ◽  
Alessia Tarditi ◽  
Caterina Mariotti ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neurotrophic Factor ◽  
Brain Derived Neurotrophic Factor
Sign in / Sign up

Export Citation Format

Share Document