scholarly journals Natural variation in sensory-motor white matter organization influences manifestations of Huntington's disease

2016 ◽  
Vol 37 (12) ◽  
pp. 4615-4628 ◽  
Author(s):  
Michael Orth ◽  
Sarah Gregory ◽  
Rachael I Scahill ◽  
Isabella SM Mayer ◽  
Lora Minkova ◽  
...  
Keyword(s):  
White Matter ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Natural Variation ◽  
Sensory Motor

scholarly journals White matter changes in Huntington’s disease and the demyelination hypothesis: a critical review

2019 ◽  
Author(s):  
chiara casella ◽  
Claudia Metzler-Baddeley ◽  
Derek Jones ◽  
Ilona Lipp
Keyword(s):  
White Matter ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neurodegenerative Disorder ◽  
Biological Properties ◽  
Disease Pathogenesis ◽  
Tissue Modeling ◽  
Cortical Grey Matter ◽  
Neuroimaging Techniques

Huntington’s disease (HD) is a genetic neurodegenerative disorder, characterised by atrophy of the neostriatum, and cortical grey matter abnormalities. White matter (WM) alterations have recently been identified as a relevant pathophysiological feature of HD, but the etiology of WM degeneration, and its role in disease pathogenesis and progression remain unclear. An increasing body of research suggests that WM changes in HD are due to alterations in myelin-associated biological processes at the cellular and molecular level. This review first discusses evidence from neurochemical studies lending support to the ‘De-myelination hypothesis’ of HD, and pointing towards a role for aberrant myelination and changes in oligodendrocytes in HD WM. Next, evidence from neuroimaging studies is reviewed, the limitations of the described methodologies are discussed and suggested interpretations of findings from published studies are challenged. Although our understanding of HD-associated pathological changes in the brain will increasingly rely on neuroimaging techniques, the shortcomings of these methodologies must not be forgotten. Advances in MRI techniques and tissue modeling will enable a better characterization of the biological properties of WM microstructure, and will allow more specific monitoring of longitudinal changes noninvasively. This, in turn, will provide insight into disease pathogenesis and progression and facilitate the identification of disease-related biomarkers and the specification of outcome measures in clinical trials.

F44 Disentangling apathy subtypes in huntington’s disease: a white matter biomarker of disease profile and progression

2018 ◽  
Author(s):  
Audrey E De Paepe ◽  
Joanna Sierpowska ◽  
Clara García-Gorro ◽  
Saül Martinez-Horta ◽  
Jesus Perez-Perez ◽  
...  
Keyword(s):  
White Matter ◽  
Huntington's Disease ◽  
Huntington’S Disease

Regional white matter change in pre-symptomatic Huntington's disease: A diffusion tensor imaging study

2005 ◽  
Vol 140 (1) ◽  
pp. 55-62 ◽  
Author(s):  
Sarah A.J. Reading ◽  
Michael A. Yassa ◽  
Arnold Bakker ◽  
Adam C. Dziorny ◽  
Lisa M. Gourley ◽  
...  
Keyword(s):  
Diffusion Tensor Imaging ◽  
White Matter ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Diffusion Tensor ◽  
Imaging Study ◽  
White Matter Change ◽  
Diffusion Tensor Imaging Study

scholarly journals In Vivo Expression of Reprogramming Factor OCT4 Ameliorates Myelination Deficits and Induces Striatal Neuroprotection in Huntington’s Disease

2020 ◽  
Author(s):  
Ji Hea Yu ◽  
Bae-Geun Nam ◽  
MinGi Kim ◽  
Jung Hwa Seo ◽  
Sung-Rae Cho
Keyword(s):  
White Matter ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Grip Strength ◽  
Cell Fate ◽  
Gabaergic Neurons ◽  
Plastic State ◽  
Qrt Pcr ◽  
Virus Serotype

Abstract Background: White matter atrophy has been shown to precede the massive loss of striatal GABAergic neurons in Huntington’s disease (HD). The HD-induced white matter atrophy is associated with motor deficits. In vivo reprogramming toward a plastic state has emerged as a new approach for treating neurological diseases. Particularly, octamer-binding transcription factor 4 (OCT4) can induce myelin repair and functional recovery. This study investigated the effects of in situ expression of reprogramming factor OCT4 on behavioral performances, neural stem cell (NSC) niche activation in the subventricular zone (SVZ) and induction of cell fate specific to the changed microenvironment of HD. Methods: R6/2 mice, a transgenic mouse model of HD, randomly received adeno-associated virus serotype 9 (AAV9)-OCT4, AAV9-Null, or phosphate-buffered saline in both lateral ventricles at 4 weeks of age. To evaluate the behavioral improvement, rotarod test and grip strength test were performed at regular intervals. To investigate the expression of oligodendrocyte progenitor cell (OPC)-related genes, real-time quantitative reverse transcription PCR (qRT-PCR) and immunohistochemistry were performed. Next, we assessed the amelioration of myelination deficits via transmission electron microscope (TEM) and magnetic resonance imaging (MRI) at 13 weeks of age. Finally, we confimed striatal neuroprotecion by qRT-PCR and confocal microscopy.Results: The AAV9-OCT4 group displayed significantly improved rotarod performance and grip strength compared to the control groups. Following AAV9-OCT4 treatment, the number of newly generated NSCs and OPCs was significantly increased in the SVZ, and the expression of OPC-related genes such as NG2, Olig2, PDGFRα, Wnt3 and myelin regulatory factor (MYRF), and glial cell-derived neuroprotective factor (GDNF) was significantly increased. Further, the amelioration of myelination deficits in the corpus callosum was observed through TEM and MRI, and striatal DARPP32+ GABAergic neurons significantly increased in the AAV9-OCT4 group.

E14 White Matter Microstructure In Huntington's Disease: 18 Month Data From The Image-hd Study

2014 ◽  
Vol 85 (Suppl 1) ◽  
pp. A40-A41
Author(s):  
G. Poudel ◽  
J. Stout ◽  
J. Dominguez ◽  
A. Churchyard ◽  
P. Chua ◽  
...  
Keyword(s):  
White Matter ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
White Matter Microstructure

scholarly journals Timing of selective basal ganglia white matter loss in premanifest Huntington’s disease

2022 ◽  
pp. 102927
Author(s):  
Paul Zeun ◽  
Peter McColgan ◽  
Thijs Dhollander ◽  
Sarah Gregory ◽  
Eileanoir B Johnson ◽  
...  
Keyword(s):  
White Matter ◽  
Basal Ganglia ◽  
Huntington's Disease ◽  
Huntington’S Disease
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