Animal models of human disease: challenges in enabling translation
P McGonigle, B Ruggeri - Biochemical pharmacology, 2014 - Elsevier
Animal models have historically played a critical role in the exploration and characterization
of disease pathophysiology, target identification, and in the in vivo evaluation of novel …
of disease pathophysiology, target identification, and in the in vivo evaluation of novel …
Cannabidiol as a promising strategy to treat and prevent movement disorders?
Movement disorders such as Parkinson's disease and dyskinesia are highly debilitating
conditions linked to oxidative stress and neurodegeneration. When available, the …
conditions linked to oxidative stress and neurodegeneration. When available, the …
[HTML][HTML] Microglia and complement mediate early corticostriatal synapse loss and cognitive dysfunction in Huntington's disease
DK Wilton, K Mastro, MD Heller, FW Gergits… - Nature medicine, 2023 - nature.com
Huntington's disease (HD) is a devastating monogenic neurodegenerative disease
characterized by early, selective pathology in the basal ganglia despite the ubiquitous …
characterized by early, selective pathology in the basal ganglia despite the ubiquitous …
A huntingtin knockin pig model recapitulates features of selective neurodegeneration in Huntington's disease
Huntington's disease (HD) is characterized by preferential loss of the medium spiny neurons
in the striatum. Using CRISPR/Cas9 and somatic nuclear transfer technology, we …
in the striatum. Using CRISPR/Cas9 and somatic nuclear transfer technology, we …
Gelation of cytoplasmic expanded CAG RNA repeats suppresses global protein synthesis
Y Pan, J Lu, X Feng, S Lu, Y Yang, G Yang… - Nature Chemical …, 2023 - nature.com
RNA molecules with the expanded CAG repeat (eCAGr) may undergo sol–gel phase
transitions, but the functional impact of RNA gelation is completely unknown. Here, we …
transitions, but the functional impact of RNA gelation is completely unknown. Here, we …
[HTML][HTML] The biological function of the Huntingtin protein and its relevance to Huntington's Disease pathology
J Schulte, JT Littleton - Current trends in neurology, 2011 - ncbi.nlm.nih.gov
Huntington's Disease is an adult-onset dominant heritable disorder characterized by
progressive psychiatric disruption, cognitive deficits, and loss of motor coordination. It is …
progressive psychiatric disruption, cognitive deficits, and loss of motor coordination. It is …
Human-to-mouse prion-like propagation of mutant huntingtin protein
I Jeon, F Cicchetti, G Cisbani, S Lee, E Li, J Bae… - Acta …, 2016 - Springer
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder of the
central nervous system (CNS) that is defined by a CAG expansion in exon 1 of the huntingtin …
central nervous system (CNS) that is defined by a CAG expansion in exon 1 of the huntingtin …
Autophagy induction by trehalose: Molecular mechanisms and therapeutic impacts
K Hosseinpour‐Moghaddam… - Journal of cellular …, 2018 - Wiley Online Library
The balance between synthesis and degradation is crucial to maintain cellular homeostasis
and different mechanisms are known to keep this balance. In this review, we will provide a …
and different mechanisms are known to keep this balance. In this review, we will provide a …
Shaping action sequences in basal ganglia circuits
Highlights•The basal ganglia are critical for chunking motor elements into action
sequences.•Consolidation of motor sequences is paralleled by decreases in neural …
sequences.•Consolidation of motor sequences is paralleled by decreases in neural …
Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington's disease
Huntington's disease (HD) is a fatal dominantly inherited neurodegenerative disorder
caused by a CAG repeat expansion leading to an elongated polyglutamine stretch in …
caused by a CAG repeat expansion leading to an elongated polyglutamine stretch in …