Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models …
PA Parone, S Da Cruz, JS Han… - Journal of …, 2013 - Soc Neuroscience
Mitochondria have been proposed as targets for toxicity in amyotrophic lateral sclerosis
(ALS), a progressive, fatal adult-onset neurodegenerative disorder characterized by the …
(ALS), a progressive, fatal adult-onset neurodegenerative disorder characterized by the …
Alternative processing of human HTT mRNA with implications for Huntington's disease therapeutics
S Fienko, C Landles, K Sathasivam, SJ McAteer… - Brain, 2022 - academic.oup.com
Huntington disease is caused by a CAG repeat expansion in exon 1 of the huntingtin gene
(HTT) that is translated into a polyglutamine stretch in the huntingtin protein (HTT). We …
(HTT) that is translated into a polyglutamine stretch in the huntingtin protein (HTT). We …
Uninterrupted CAG repeat drives striatum-selective transcriptionopathy and nuclear pathogenesis in human Huntingtin BAC mice
X Gu, J Richman, P Langfelder, N Wang, S Zhang… - Neuron, 2022 - cell.com
In Huntington's disease (HD), the uninterrupted CAG repeat length, but not the
polyglutamine length, predicts disease onset. However, the underlying pathobiology …
polyglutamine length, predicts disease onset. However, the underlying pathobiology …
The role of the immune system in Huntington's disease
G Ellrichmann, C Reick, C Saft… - Journal of Immunology …, 2013 - Wiley Online Library
Huntington's disease (HD) is characterized by a progressive course of disease until death
15–20 years after the first symptoms occur and is caused by a mutation with expanded CAG …
15–20 years after the first symptoms occur and is caused by a mutation with expanded CAG …
Genome-wide loss of 5-hmC is a novel epigenetic feature of Huntington's disease
F Wang, Y Yang, X Lin, JQ Wang, YS Wu… - Human molecular …, 2013 - academic.oup.com
5-Hydroxymethylcytosine (5-hmC) may represent a new epigenetic modification of cytosine.
While the dynamics of 5-hmC during neurodevelopment have recently been reported, little is …
While the dynamics of 5-hmC during neurodevelopment have recently been reported, little is …
Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and …
The development of animal models of Huntington disease (HD) has enabled studies that
help define the molecular aberrations underlying the disease. The BACHD and YAC128 …
help define the molecular aberrations underlying the disease. The BACHD and YAC128 …
Putting proteins in their place: palmitoylation in Huntington disease and other neuropsychiatric diseases
FB Young, SL Butland, SS Sanders, LM Sutton… - Progress in …, 2012 - Elsevier
Post-translational modification of proteins by the lipid palmitate is critical for protein
localization and function. Palmitoylation is regulated by the opposing enzymes palmitoyl …
localization and function. Palmitoylation is regulated by the opposing enzymes palmitoyl …
Neuroinflammation in Huntington's disease: From animal models to clinical therapeutics
Q Jia, S Li, XJ Li, P Yin - Frontiers in Immunology, 2022 - frontiersin.org
Huntington's disease (HD) is a progressive neurodegenerative disease characterized by
preferential loss of neurons in the striatum in patients, which leads to motor and cognitive …
preferential loss of neurons in the striatum in patients, which leads to motor and cognitive …
A transgenic minipig model of Huntington's disease
M Baxa, M Hruska-Plochan, S Juhas… - Journal of …, 2013 - content.iospress.com
Background: Some promising treatments for Huntington's disease (HD) may require pre-
clinical testing in large animals. Minipig is a suitable species because of its large …
clinical testing in large animals. Minipig is a suitable species because of its large …
The huntingtin N17 domain is a multifunctional CRM1 and Ran-dependent nuclear and cilial export signal
T Maiuri, T Woloshansky, J Xia… - Human molecular …, 2013 - academic.oup.com
The first 17 amino acids of Huntington's disease (HD) protein, huntingtin, comprise an
amphipathic alpha-helical domain that can target huntingtin to the endoplasmic reticulum …
amphipathic alpha-helical domain that can target huntingtin to the endoplasmic reticulum …