Clinical neurology and epidemiology of the major neurodegenerative diseases
MG Erkkinen, MO Kim… - Cold Spring Harbor …, 2018 - cshperspectives.cshlp.org
Neurodegenerative diseases are a common cause of morbidity and cognitive impairment in
older adults. Most clinicians who care for the elderly are not trained to diagnose these …
older adults. Most clinicians who care for the elderly are not trained to diagnose these …
Dopamine imbalance in Huntington's disease: a mechanism for the lack of behavioral flexibility
Dopamine (DA) plays an essential role in the control of coordinated movements. Alterations
in DA balance in the striatum lead to pathological conditions such as Parkinson's and …
in DA balance in the striatum lead to pathological conditions such as Parkinson's and …
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 …
RAN translation in Huntington disease
M Banez-Coronel, F Ayhan, AD Tarabochia, T Zu… - Neuron, 2015 - cell.com
Huntington disease (HD) is caused by a CAG⋅ CTG expansion in the huntingtin (HTT) gene.
While most research has focused on the HTT polyGln-expansion protein, we demonstrate …
While most research has focused on the HTT polyGln-expansion protein, we demonstrate …
RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model
SQ Harper, PD Staber, X He… - Proceedings of the …, 2005 - National Acad Sciences
Huntington's disease (HD) is a fatal, dominant neurogenetic disorder. HD results from
polyglutamine repeat expansion (CAG codon, Q) in exon 1 of HD, conferring a toxic gain of …
polyglutamine repeat expansion (CAG codon, Q) in exon 1 of HD, conferring a toxic gain of …
The clinical and genetic features of Huntington disease
A Sturrock, BR Leavitt - Journal of geriatric psychiatry and …, 2010 - journals.sagepub.com
Huntington disease (HD) is a dominantly inherited neurodegenerative disorder that usually
presents in adulthood with characteristic motor and cognitive features and with variable and …
presents in adulthood with characteristic motor and cognitive features and with variable and …
Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's …
An expanded polyglutamine (polyQ) stretch in the protein huntingtin (htt) induces self-
aggregation into inclusion bodies (IBs) and causes Huntington's disease (HD). Defining …
aggregation into inclusion bodies (IBs) and causes Huntington's disease (HD). Defining …
Mitochondrial dynamics and quality control in Huntington's disease
P Guedes-Dias, BR Pinho, TR Soares… - Neurobiology of …, 2016 - Elsevier
Huntington's disease (HD) is an inherited neurodegenerative disorder caused by
polyglutamine expansion mutations in the huntingtin protein. Despite its ubiquitous …
polyglutamine expansion mutations in the huntingtin protein. Despite its ubiquitous …
Mechanism of ER stress-induced brain damage by IP3 receptor
Deranged Ca 2+ signaling and an accumulation of aberrant proteins cause endoplasmic
reticulum (ER) stress, which is a hallmark of cell death implicated in many …
reticulum (ER) stress, which is a hallmark of cell death implicated in many …
Alterations in cortical excitation and inhibition in genetic mouse models of Huntington's disease
DM Cummings, VM André, BO Uzgil… - Journal of …, 2009 - Soc Neuroscience
Previously, we identified progressive alterations in spontaneous EPSCs and IPSCs in the
striatum of the R6/2 mouse model of Huntington's disease (HD). Medium-sized spiny …
striatum of the R6/2 mouse model of Huntington's disease (HD). Medium-sized spiny …