The neuropathology of Huntington's disease

HJ Waldvogel, EH Kim, LJ Tippett… - … of Huntington's disease …, 2015 - Springer
The basal ganglia are a highly interconnected set of subcortical nuclei and major atrophy in
one or more regions may have major effects on other regions of the brain. Therefore, the …

Genetics and neuropathology of Huntington's disease

A Reiner, I Dragatsis, P Dietrich - International review of neurobiology, 2011 - Elsevier
Huntington's disease (HD) is an autosomal dominant progressive neurodegenerative
disorder that prominently affects the basal ganglia, leading to affective, cognitive, behavioral …

Regional specificity of brain atrophy in Huntington's disease

GM Halliday, DA McRitchie, V Macdonald… - Experimental …, 1998 - Elsevier
The present study analyzes the relationship between cortical and subcortical brain volumes
in patients with Huntington's disease. The brains of seven patients with a clinical diagnosis …

Differential loss of striatal projection systems in Huntington's disease: a quantitative immunohistochemical study

YP Deng, RL Albin, JB Penney, AB Young… - Journal of chemical …, 2004 - Elsevier
Prior studies suggest differences exist among striatal projection neuron types in their
vulnerability to Huntington's disease (HD). In the present study, we immunolabeled the fibers …

[HTML][HTML] Pathological cell-cell interactions elicited by a neuropathogenic form of mutant Huntingtin contribute to cortical pathogenesis in HD mice

X Gu, C Li, W Wei, V Lo, S Gong, SH Li, T Iwasato… - Neuron, 2005 - cell.com
Expanded polyglutamine (polyQ) proteins in Huntington's disease (HD) as well as other
polyQ disorders are known to elicit a variety of intracellular toxicities, but it remains unclear …

Chemical anatomy of striatal interneurons in normal individuals and in patients with Huntington's disease

F Cicchetti, L Prensa, Y Wu, A Parent - Brain research reviews, 2000 - Elsevier
This paper reviews the major anatomical and chemical features of the various types of
interneurons in the human striatum, as detected by immunostaining procedures applied to …

Cellular localization of huntingtin in striatal and cortical neurons in rats: lack of correlation with neuronal vulnerability in Huntington's disease

FR Fusco, Q Chen, WJ Lamoreaux… - Journal of …, 1999 - Soc Neuroscience
Immunohistochemistry and single-cell RT-PCR were used to characterize the localization of
huntingtin and/or its mRNA in the major types of striatal neurons and in corticostriatal …

Disrupted striatal neuron inputs and outputs in Huntington's disease

A Reiner, YP Deng - CNS neuroscience & therapeutics, 2018 - Wiley Online Library
Huntington's disease (HD) is a hereditary progressive neurodegenerative disorder caused
by a CAG repeat expansion in the gene coding for the protein huntingtin, resulting in a …

The selective vulnerability of striatopallidal neurons

IJ Mitchell, AJ Cooper, MR Griffiths - Progress in neurobiology, 1999 - Elsevier
The different types of striatal neuron show a range of vulnerabilities to a variety of insults.
This can be clearly seen in Huntington's disease where a well mapped pattern of …

Striatal parvalbuminergic neurons are lost in Huntington's disease: implications for dystonia

A Reiner, E Shelby, H Wang, Z DeMarch… - Movement …, 2013 - Wiley Online Library
Although dystonia represents a major source of motor disability in Huntington's disease
(HD), its pathophysiology remains unknown. Because recent animal studies indicate that …