The impact of an intense religious experience on motor symptoms in Huntington's disease

2008 ◽  
Vol 24 (3) ◽  
pp. 473-474 ◽  
Author(s):  
Jose Luis López-Sendón Moreno ◽  
Justo Garcia de Yebenes
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Religious Experience ◽  
Motor Symptoms ◽  
The Impact

Abnormal Spinal Cord Myelination due to Oligodendrocyte Dysfunction in a Model of Huntington’s Disease

2021 ◽  
pp. 1-8
Author(s):  
Costanza Ferrari Bardile ◽  
Harwin Sidik ◽  
Reynard Quek ◽  
Nur Amirah Binte Mohammad Yusof ◽  
Marta Garcia-Miralles ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Oligodendrocyte Progenitor Cells ◽  
Wild Type ◽  
Specific Expression ◽  
Relative Contribution ◽  
Related Proteins ◽  
The Impact ◽  
Cervical Area

Background: The relative contribution of grey matter (GM) and white matter (WM) degeneration to the progressive brain atrophy in Huntington’s disease (HD) has been well studied. The pathology of the spinal cord in HD is comparatively less well documented. Objective: We aim to characterize spinal cord WM abnormalities in a mouse model of HD and evaluate whether selective removal of mutant huntingtin (mHTT) from oligodendroglia rescues these deficits. Methods: Histological assessments were used to determine the area of GM and WM in the spinal cord of 12-month-old BACHD mice, while electron microscopy was used to analyze myelin fibers in the cervical area of the spinal cord. To investigate the impact of inactivation of mHTT in oligodendroglia on these measures, we used the previously described BACHDxNG2Cre mouse line where mHTT is specifically reduced in oligodendrocyte progenitor cells. Results: We show that spinal GM and WM areas are significantly atrophied in HD mice compared to wild-type controls. We further demonstrate that specific reduction of mHTT in oligodendroglial cells rescues the atrophy of spinal cord WM, but not GM, observed in HD mice. Inactivation of mHTT in oligodendroglia had no effect on the density of oligodendroglial cells but enhanced the expression of myelin-related proteins in the spinal cord. Conclusion: Our findings demonstrate that the myelination abnormalities observed in brain WM structures in HD extend to the spinal cord and suggest that specific expression of mHTT in oligodendrocytes contributes to such abnormalities.

66. Effects of cranio-spinal tDCS in the treatment of cognitive and motor symptoms in early Huntington’s Disease

2016 ◽  
Vol 127 (12) ◽  
pp. e339
Author(s):  
L. Parenti ◽  
D. Barloscio ◽  
M. Bartolotta ◽  
A. Di Rollo ◽  
F. Sartucci ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Motor Symptoms

scholarly journals Characterising Upper Limb Movements in Huntington's Disease and the Impact of Restricted Visual Cues

PLoS ONE ◽  
2015 ◽  
Vol 10 (8) ◽  
pp. e0133709 ◽  
Author(s):  
Jessica Despard ◽  
Anne-Marie Ternes ◽  
Bleydy Dimech-Betancourt ◽  
Govinda Poudel ◽  
Andrew Churchyard ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Upper Limb ◽  
Visual Cues ◽  
Limb Movements ◽  
Upper Limb Movements ◽  
The Impact

scholarly journals Time-Restricted Feeding Improves Circadian Dysfunction as well as Motor Symptoms in the Q175 Mouse Model of Huntington’s Disease

eNeuro ◽  
2018 ◽  
Vol 5 (1) ◽  
pp. ENEURO.0431-17.2017 ◽  
Author(s):  
Huei-Bin Wang ◽  
Dawn H. Loh ◽  
Daniel S. Whittaker ◽  
Tamara Cutler ◽  
David Howland ◽  
...  
Keyword(s):  
Mouse Model ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Motor Symptoms ◽  
Restricted Feeding ◽  
Circadian Dysfunction

scholarly journals The impact of the gut microbiota on Huntington's disease mice

IBRO Reports ◽  
2019 ◽  
Vol 6 ◽  
pp. S370-S371
Author(s):  
Carolina De Moura Gubert ◽  
Geraldine Kong ◽  
Jamie Liew ◽  
Chloe Love ◽  
Thibault Renoir ◽  
...  
Keyword(s):  
Gut Microbiota ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
The Impact

scholarly journals IKKβ slows Huntington’s disease progression in R6/1 mice

2019 ◽  
Vol 116 (22) ◽  
pp. 10952-10961 ◽  
Author(s):  
Joseph Ochaba ◽  
Gianna Fote ◽  
Marketta Kachemov ◽  
Soe Thein ◽  
Sylvia Y. Yeung ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuronal Degeneration ◽  
Neuronal Injury ◽  
Therapeutic Interventions ◽  
Striatal Neurons ◽  
Microglial Response ◽  
Relationship Of ◽  
The Impact

Neuroinflammation is an important contributor to neuronal pathology and death in neurodegenerative diseases and neuronal injury. Therapeutic interventions blocking the activity of the inflammatory kinase IKKβ, a key regulator of neuroinflammatory pathways, is protective in several animal models of neurodegenerative disease and neuronal injury. In Huntington’s disease (HD), however, significant questions exist as to the impact of blocking or diminishing the activity of IKKβ on HD pathology given its potential role in Huntingtin (HTT) degradation. In cell culture, IKKβ phosphorylates HTT serine (S) 13 and activates HTT degradation, a process that becomes impaired with polyQ expansion. To investigate the in vivo relationship of IKKβ to HTT S13 phosphorylation and HD progression, we crossed conditional tamoxifen-inducible IKKβ knockout mice with R6/1 HD mice. Behavioral assays in these mice showed a significant worsening of HD pathological phenotypes. The increased behavioral pathology correlated with reduced levels of endogenous mouse full-length phospho-S13 HTT, supporting the importance of IKKβ in the phosphorylation of HTT S13 in vivo. Notably, many striatal autophagy genes were up-regulated in HD vs. control mice; however, IKKβ knockout partially reduced this up-regulation in HD, increased striatal neurodegeneration, and enhanced an activated microglial response. We propose that IKKβ is protective in striatal neurons early in HD progression via phosphorylation of HTT S13. As IKKβ is also required for up-regulation of some autophagy genes and HTT is a scaffold for selective autophagy, IKKβ may influence autophagy through multiple mechanisms to maintain healthy striatal function, thereby reducing neuronal degeneration to slow HD onset.

The Impact of Juvenile Huntington's Disease on the Family

2008 ◽  
Vol 13 (1) ◽  
pp. 5-16 ◽  
Author(s):  
Helen M. Brewer ◽  
Virginia Eatough ◽  
Jonathan A. Smith ◽  
Cath A. Stanley ◽  
Neil W. Glendinning ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
The Family ◽  
Juvenile Huntington’S Disease ◽  
The Impact

F46 Relationship between distinct motor symptoms and apathy in huntington’s disease: clues to mechanism

2018 ◽  
Author(s):  
Akshay Nair ◽  
N Ahmad Aziz ◽  
Robb Rutledge ◽  
Geraint Rees ◽  
Sarah J Tabrizi
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Motor Symptoms

scholarly journals The impact of occipital lobe cortical thickness on cognitive task performance: An investigation in Huntington's Disease

2015 ◽  
Vol 79 ◽  
pp. 138-146 ◽  
Author(s):  
Eileanoir B. Johnson ◽  
Elin M. Rees ◽  
Izelle Labuschagne ◽  
Alexandra Durr ◽  
Blair R. Leavitt ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Task Performance ◽  
Cortical Thickness ◽  
Cognitive Task ◽  
Occipital Lobe ◽  
Cognitive Task Performance ◽  
The Impact

What is the impact of education on Huntington's disease?

2011 ◽  
Vol 26 (8) ◽  
pp. 1489-1495 ◽  
Author(s):  
Jose Luis López-Sendón ◽  
Ana Royuela ◽  
Patricia Trigo ◽  
Michael Orth ◽  
Herwig Lange ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
The Impact
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