scholarly journals Lessons Learned from the Transgenic Huntington's Disease Rats

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Rinske Vlamings ◽  
Dagmar H. Zeef ◽  
Marcus L. F. Janssen ◽  
Mayke Oosterloo ◽  
Frederic Schaper ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Nonhuman Primates ◽  
Neuropsychiatric Symptoms ◽  
Relevant Literature ◽  
Lessons Learned ◽  
Cag Repeats ◽  
Transgenic Rat ◽  
Cdna Fragment ◽  
Transgenic Rat Model

Huntington's disease (HD) is a fatal inherited disorder leading to selective neurodegeneration and neuropsychiatric symptoms. Currently, there is no treatment to slow down or to stop the disease. There is also no therapy to effectively reduce the symptoms. In the investigation of novel therapies, different animal models of Huntington's disease, varying from insects to nonhuman primates, have been created and used. Few years ago, the first transgenic rat model of HD, carrying a truncated huntingtin cDNA fragment with 51 CAG repeats under control of the native rathuntingtinpromoter, was introduced. We have been using this animal model in our research and review here our experience with the behavioural, neurophysiological, and histopathological phenotype of the transgenic Huntington's disease rats with relevant literature.

scholarly journals Motivational Impairment is Accompanied by Corticoaccumbal Dysfunction in the BACHD-Tg5 Rat Model of Huntington’s Disease

2019 ◽  
Vol 29 (11) ◽  
pp. 4763-4774 ◽  
Author(s):  
Natalie E Zlebnik ◽  
Iness Gildish ◽  
Thibaut Sesia ◽  
Aurelie Fitoussi ◽  
Ellen A Cole ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rat Model ◽  
Neuropsychiatric Symptoms ◽  
Transgenic Rat ◽  
Psychiatric Disturbances ◽  
Reward Motivation ◽  
Motor Abnormalities ◽  
Insight Into ◽  
Transgenic Rat Model

Abstract Neuropsychiatric symptoms, such as avolition, apathy, and anhedonia, precede the onset of debilitating motor symptoms in Huntington’s disease (HD), and their development may give insight into early disease progression and treatment. However, the neuronal and circuit mechanisms of premanifest HD pathophysiology are not well-understood. Here, using a transgenic rat model expressing the full-length human mutant HD gene, we find early and profound deficits in reward motivation in the absence of gross motor abnormalities. These deficits are accompanied by significant and progressive dysfunction in corticostriatal processing and communication among brain areas critical for reward-driven behavior. Together, our results define early corticostriatal dysfunction as a possible pathogenic contributor to psychiatric disturbances and may help identify potential pharmacotherapeutic targets for the treatment of HD.

scholarly journals Transplants of Adult Mesenchymal and Neural Stem Cells Provide Neuroprotection and Behavioral Sparing in a Transgenic Rat Model of Huntington's Disease

Stem Cells ◽  
2014 ◽  
Vol 32 (2) ◽  
pp. 500-509 ◽  
Author(s):  
Julien Rossignol ◽  
Kyle Fink ◽  
Kendra Davis ◽  
Steven Clerc ◽  
Andrew Crane ◽  
...  
Keyword(s):  
Stem Cells ◽  
Neural Stem Cells ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rat Model ◽  
Transgenic Rat ◽  
Transgenic Rat Model

C08 Set-shifting Deficits In The Pre-symptomatic Bachd Transgenic Rat Model Of Huntington's Disease

2014 ◽  
Vol 85 (Suppl 1) ◽  
pp. A27-A27
Author(s):  
M. Sosti ◽  
S. Martinez-Horta ◽  
J. Perez Perez ◽  
F. Arenas ◽  
J. Kulisevsky
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rat Model ◽  
Transgenic Rat ◽  
Set Shifting ◽  
Transgenic Rat Model

Profiles of motor and cognitive impairment in the transgenic rat model of Huntington's disease

2012 ◽  
Vol 88 (2-3) ◽  
pp. 223-236 ◽  
Author(s):  
Steven A. Fielding ◽  
Simon P. Brooks ◽  
Alexander Klein ◽  
Zubeyde Bayram-Weston ◽  
Lesley Jones ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rat Model ◽  
Transgenic Rat ◽  
Transgenic Rat Model

Altered expression of dopaminergic cell fate regulating genes prior to manifestation of symptoms in a transgenic rat model of Huntington's disease

2019 ◽  
Vol 1712 ◽  
pp. 101-108 ◽  
Author(s):  
Gemma Huguet ◽  
Yasin Temel ◽  
Elisabet Kádár ◽  
Sylvana Pol ◽  
Joao Casaca-Carreira ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cell Fate ◽  
Rat Model ◽  
Transgenic Rat ◽  
Altered Expression ◽  
Dopaminergic Cell ◽  
Transgenic Rat Model

Age-dependent gene expression profile and protein expression in a transgenic rat model of Huntington's disease

2008 ◽  
Vol 2 (12) ◽  
pp. 1638-1650 ◽  
Author(s):  
Huu Phuc Nguyen ◽  
Silke Metzger ◽  
Carsten Holzmann ◽  
Dirk Koczan ◽  
Hans-Jürgen Thiesen ◽  
...  
Keyword(s):  
Gene Expression ◽  
Protein Expression ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Gene Expression Profile ◽  
Expression Profile ◽  
Rat Model ◽  
Transgenic Rat ◽  
Age Dependent ◽  
Transgenic Rat Model

scholarly journals Huntington's disease research and practice: reflections on the journey made and lessons learned

2011 ◽  
Vol 23 (6) ◽  
pp. 851-857 ◽  
Author(s):  
Anita M.Y. Goh ◽  
Edmond Chiu
Keyword(s):  
Neurodegenerative Disease ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuropsychiatric Symptoms ◽  
Current Treatment ◽  
Lessons Learned ◽  
Monogenic Disorder ◽  
European Origin ◽  
Common Causes ◽  
The Usa

Knowledge about some of the rarer causes of dementia is now quite advanced (Lautenschlager and Martins, 2005), which can in turn inform other more common causes of dementia. Such is the case with the monogenic disorder of Huntington's disease (HD) when compared to, say, Alzheimer's disease (AD). HD is an autosomal dominant hereditary neurodegenerative disease, which involves the basal ganglia, its connections to the frontal lobe and related neural circuits. The onset of HD is typically in mid-life (but onset can range from childhood to old age), with motor, cognitive and neuropsychiatric symptoms. There is currently no cure for this devastating and inevitably fatal neurodegenerative disease, with current treatment approaches being solely symptomatic. The highest frequencies of HD are found in Europe and in those countries whose populations are of predominately European origin such as the USA and Australia (approximately 1 case per 10,000 people).

Early cognitive dysfunction in the HD 51 CAG transgenic rat model of Huntington's disease.

2012 ◽  
Vol 126 (3) ◽  
pp. 479-487 ◽  
Author(s):  
Kyle D. Fink ◽  
Julien Rossignol ◽  
Andrew T. Crane ◽  
Kendra K. Davis ◽  
Angela M. Bavar ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Dysfunction ◽  
Rat Model ◽  
Transgenic Rat ◽  
Transgenic Rat Model

scholarly journals AAV1/2-mediated BDNF gene therapy in a transgenic rat model of Huntington’s disease

Gene Therapy ◽  
2015 ◽  
Vol 23 (3) ◽  
pp. 283-295 ◽  
Author(s):  
B Connor ◽  
Y Sun ◽  
D von Hieber ◽  
S K Tang ◽  
K S Jones ◽  
...  
Keyword(s):  
Gene Therapy ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rat Model ◽  
Transgenic Rat ◽  
Bdnf Gene ◽  
Transgenic Rat Model
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