Huntington's disease: mechanisms of pathogenesis and therapeutic strategies

M Jimenez-Sanchez, F Licitra… - Cold Spring …, 2017 - perspectivesinmedicine.cshlp.org
Huntington's disease is a late-onset neurodegenerative disease caused by a CAG
trinucleotide repeat in the gene encoding the huntingtin protein. Despite its well-defined …

Huntington disease: natural history, biomarkers and prospects for therapeutics

CA Ross, EH Aylward, EJ Wild, DR Langbehn… - Nature Reviews …, 2014 - nature.com
Huntington disease (HD) can be seen as a model neurodegenerative disorder, in that it is
caused by a single genetic mutation and is amenable to predictive genetic testing, with …

Mitochondria and neuroplasticity

A Cheng, Y Hou, MP Mattson - ASN neuro, 2010 - journals.sagepub.com
The production of neurons from neural progenitor cells, the growth of axons and dendrites
and the formation and reorganization of synapses are examples of neuroplasticity. These …

Pathophysiology of Huntington's disease: time-dependent alterations in synaptic and receptor function

LA Raymond, VM André, C Cepeda, CM Gladding… - Neuroscience, 2011 - Elsevier
Huntington's disease (HD) is a progressive, fatal neurological condition caused by an
expansion of CAG (glutamine) repeats in the coding region of the Huntington gene. To date …

[HTML][HTML] Early synaptic pathophysiology in neurodegeneration: insights from Huntington's disease

AJ Milnerwood, LA Raymond - Trends in neurosciences, 2010 - cell.com
Investigations of synaptic transmission and plasticity in mouse models of Huntington's
disease (HD) demonstrate neuronal dysfunction long before the onset of classical disease …

[HTML][HTML] Cortical and striatal circuits in Huntington's disease

S Blumenstock, I Dudanova - Frontiers in neuroscience, 2020 - frontiersin.org
Huntington's disease (HD) is a hereditary neurodegenerative disorder that typically
manifests in midlife with motor, cognitive, and/or psychiatric symptoms. The disease is …

The corticostriatal pathway in Huntington's disease

C Cepeda, N Wu, VM André, DM Cummings… - Progress in …, 2007 - Elsevier
The corticostriatal pathway provides most of the excitatory glutamatergic input into the
striatum and it plays an important role in the development of the phenotype of Huntington's …

Alterations in synaptic function and plasticity in Huntington disease

AI Smith‐Dijak, MD Sepers… - Journal of …, 2019 - Wiley Online Library
Huntington disease (HD) is an inherited neurodegenerative disorder caused by an
expansion of the CAG repeat region in the first exon of the huntingtin gene …

[HTML][HTML] Huntington's disease: can mice lead the way to treatment?

ZR Crook, D Housman - Neuron, 2011 - cell.com
Mouse models for Huntington's Disease (HD) and HD patients demonstrate motor and
behavioral dysfunctions, such as progressive loss of coordination and memory, and share …

The role of Smo-Shh/Gli signaling activation in the prevention of neurological and ageing disorders

A Prajapati, S Mehan, Z Khan - Biogerontology, 2023 - Springer
Sonic hedgehog (Shh) signaling is an essential central nervous system (CNS) pathway
involved during embryonic development and later life stages. Further, it regulates cell …