scholarly journals Correction to Poly(trehalose) Nanoparticles Prevent Amyloid Aggregation and Suppress Polyglutamine Aggregation in a Huntington’s Disease Model Mouse

2019 ◽  
Vol 11 (33) ◽  
pp. 30508-30508
Author(s):  
Koushik Debnath ◽  
Nibedita Pradhan ◽  
Brijesh Kumar Singh ◽  
Nihar R. Jana ◽  
Nikhil R. Jana
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Model ◽  
Amyloid Aggregation ◽  
Model Mouse ◽  
Polyglutamine Aggregation

scholarly journals Poly(trehalose) Nanoparticles Prevent Amyloid Aggregation and Suppress Polyglutamine Aggregation in a Huntington’s Disease Model Mouse

2017 ◽  
Vol 9 (28) ◽  
pp. 24126-24139 ◽  
Author(s):  
Koushik Debnath ◽  
Nibedita Pradhan ◽  
Brijesh Kumar Singh ◽  
Nihar R. Jana ◽  
Nikhil R. Jana
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Model ◽  
Amyloid Aggregation ◽  
Model Mouse ◽  
Polyglutamine Aggregation

I23 Tracking transplanted human foetal cells using bioluminescence imaging in a huntington’s disease model

2018 ◽  
Author(s):  
Mutwakil Abdulla ◽  
Amit Jathoul ◽  
Ngoc-Nga Vinh ◽  
Ying Zhu ◽  
David Williams ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Bioluminescence Imaging ◽  
Disease Model

scholarly journals Mitochondrial Respiration Changes in R6/2 Huntington’s Disease Model Mice during Aging in a Brain Region Specific Manner

2020 ◽  
Vol 21 (15) ◽  
pp. 5412 ◽  
Author(s):  
Johannes Burtscher ◽  
Alba Di Pardo ◽  
Vittorio Maglione ◽  
Christoph Schwarzer ◽  
Ferdinando Squitieri
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Model ◽  
Brain Regions ◽  
Future Research ◽  
Adult Mice ◽  
Age Related ◽  
Developmental Shift ◽  
Regional Vulnerability ◽  
Oxygen Conditions

Mitochondrial dysfunction is crucially involved in aging and neurodegenerative diseases, such as Huntington’s Disease (HD). How mitochondria become compromised in HD is poorly understood but instrumental for the development of treatments to prevent or reverse resulting deficits. In this paper, we investigate whether oxidative phosphorylation (OXPHOS) differs across brain regions in juvenile as compared to adult mice and whether such developmental changes might be compromised in the R6/2 mouse model of HD. We study OXPHOS in the striatum, hippocampus, and motor cortex by high resolution respirometry in female wild-type and R6/2 mice of ages corresponding to pre-symptomatic and symptomatic R6/2 mice. We observe a developmental shift in OXPHOS-control parameters that was similar in R6/2 mice, except for cortical succinate-driven respiration. While the LEAK state relative to maximal respiratory capacity was reduced in adult mice in all analyzed brain regions, succinate-driven respiration was reduced only in the striatum and cortex, and NADH-driven respiration was higher as compared to juvenile mice only in the striatum. We demonstrate age-related changes in respirational capacities of different brain regions with subtle deviations in R6/2 mice. Uncovering in situ oxygen conditions and potential substrate limitations during aging and HD disease progression are interesting avenues for future research to understand brain-regional vulnerability in HD.

Striatal Cholesterol Precursors Are Altered with Age in Female Huntington’s Disease Model Mice

2019 ◽  
Vol 8 (2) ◽  
pp. 161-169 ◽  
Author(s):  
Anna C. Pfalzer ◽  
Phillip A. Wages ◽  
Ned A. Porter ◽  
Aaron B. Bowman
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Model ◽  
Cholesterol Precursors

scholarly journals Cystamine increases l-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation

2004 ◽  
Vol 91 (2) ◽  
pp. 413-422 ◽  
Author(s):  
Jonathan H. Fox ◽  
David S. Barber ◽  
Bhupinder Singh ◽  
Birgit Zucker ◽  
Mary K. Swindell ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Transgenic Mouse ◽  
Mouse Brain ◽  
Polyglutamine Aggregation

scholarly journals Towards humane end points: behavioural changes precede clinical signs of disease in a Huntington's disease model

2008 ◽  
Vol 275 (1645) ◽  
pp. 1865-1874 ◽  
Author(s):  
Kate Littin ◽  
Abraham Acevedo ◽  
William Browne ◽  
Joanne Edgar ◽  
Mike Mendl ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Model ◽  
Clinical Signs ◽  
End Points ◽  
Behavioural Changes
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