[HTML][HTML] Targeting mitochondrial network disorganization is protective in C. elegans models of Huntington's disease

E Machiela, PD Rudich, A Traa, U Anglas… - Aging and …, 2021 - ncbi.nlm.nih.gov
Huntington's disease (HD) is an adult-onset neurodegenerative disease caused by a
trinucleotide CAG repeat expansion in the HTT gene. While the pathogenesis of HD is …

[HTML][HTML] Molecular mechanisms underlying muscle wasting in Huntington's disease

M Bozzi, F Sciandra - International journal of molecular sciences, 2020 - mdpi.com
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by
pathogenic expansions of the triplet cytosine-adenosine-guanosine (CAG) within the …

Natural history of disease in the YAC128 mouse reveals a discrete signature of pathology in Huntington disease

JB Carroll, JP Lerch, S Franciosi, A Spreeuw… - Neurobiology of …, 2011 - Elsevier
Abstract Models of Huntington disease (HD) recapitulate some neuropathological features of
the disease. However, a global natural history of neuroanatomy in a mouse expressing full …

[HTML][HTML] Induced pluripotent stem cell-derived neural stem cell transplantations reduced behavioral deficits and ameliorated neuropathological changes in YAC128 …

A Al-Gharaibeh, R Culver, AN Stewart… - Frontiers in …, 2017 - frontiersin.org
Huntington's disease (HD) is a genetic neurodegenerative disorder characterized by
neuronal loss and motor dysfunction. Although there is no effective treatment, stem cell …

[HTML][HTML] Characterisation of immune cell function in fragment and full-length Huntington's disease mouse models

U Träger, R Andre, A Magnusson-Lind, JRC Miller… - Neurobiology of …, 2015 - Elsevier
Inflammation is a growing area of research in neurodegeneration. In Huntington's disease
(HD), a fatal inherited neurodegenerative disease caused by a CAG-repeat expansion in the …

Progression of basal ganglia pathology in heterozygous Q175 knock‐in Huntington's disease mice

Y Deng, H Wang, M Joni, R Sekhri… - Journal of Comparative …, 2021 - Wiley Online Library
We used behavioral testing and morphological methods to detail the progression of basal
ganglia neuron type‐specific pathology and the deficits stemming from them in male …

[HTML][HTML] Rodent Models of Huntington's Disease: An Overview

G Nittari, P Roy, I Martinelli, V Bellitto, D Tomassoni… - Biomedicines, 2023 - mdpi.com
Huntington's disease (HD) is an autosomal-dominant inherited neurological disorder caused
by a genetic mutation in the IT15 gene. This neurodegenerative disorder is caused by a …

Functional differences between direct and indirect striatal output pathways in Huntington's disease

L Galvan, VM André, EA Wang… - Journal of …, 2012 - content.iospress.com
There is morphological evidence for differential alterations in striatal medium-sized spiny
neurons (MSNs) giving rise to the direct and indirect output pathways in Huntington's …

Antisense oligonucleotide-mediated correction of transcriptional dysregulation is correlated with behavioral benefits in the YAC128 mouse model of Huntington's …

LM Stanek, W Yang, S Angus, PS Sardi… - Journal of …, 2013 - content.iospress.com
Background: Huntington's disease (HD) is a neurological disorder caused by mutations in
the huntingtin (HTT) gene, the product of which leads to selective and progressive neuronal …

[HTML][HTML] The role of voltage-gated calcium channels in basal ganglia neurodegenerative disorders

BHM Correa, CR Moreira, ME Hildebrand… - Current …, 2023 - ncbi.nlm.nih.gov
Abstract Calcium (Ca 2+) plays a central role in regulating many cellular processes and
influences cell survival. Several mechanisms can disrupt Ca 2+ homeostasis to trigger cell …