Oxidative stress and Huntington's disease: The good, the bad, and the ugly

A Kumar, RR Ratan - Journal of Huntington's disease, 2016 - content.iospress.com
Redox homeostasis is crucial for proper cellular functions, including receptor tyrosine kinase
signaling, protein folding, and xenobiotic detoxification. Under basal conditions, there is a …

A systematic review of transcriptional dysregulation in Huntington's disease studied by RNA sequencing

B Malla, X Guo, G Senger, Z Chasapopoulou… - Frontiers in …, 2021 - frontiersin.org
Huntington's disease (HD) is a chronic neurodegenerative disorder caused by an expansion
of polyglutamine repeats in exon 1 of the Huntingtin gene. Transcriptional dysregulation …

Developmental alterations in Huntington's disease neural cells and pharmacological rescue in cells and mice

Nature neuroscience, 2017 - nature.com
Neural cultures derived from Huntington's disease (HD) patient-derived induced pluripotent
stem cells were used for'omics' analyses to identify mechanisms underlying …

HD and SCA1: Tales from two 30-year journeys since gene discovery

LM Thompson, HT Orr - Neuron, 2023 - cell.com
One of the more transformative findings in human genetics was the discovery that the
expansion of unstable nucleotide repeats underlies a group of inherited neurological …

[HTML][HTML] Targeting super-enhancers for disease treatment and diagnosis

HY Shin - Molecules and cells, 2018 - Elsevier
The transcriptional regulation of genes determines the fate of animal cell differentiation and
subsequent organ development. With the recent progress in genome-wide technologies, the …

Reinstating plasticity and memory in a tauopathy mouse model with an acetyltransferase activator

S Chatterjee, R Cassel… - EMBO Molecular …, 2018 - embopress.org
Chromatin acetylation, a critical regulator of synaptic plasticity and memory processes, is
thought to be altered in neurodegenerative diseases. Here, we demonstrate that spatial …

[HTML][HTML] Sphingolipids and impaired hypoxic stress responses in Huntington disease

J Burtscher, G Pepe, N Maharjan, N Riguet… - Progress in lipid …, 2023 - Elsevier
Huntington disease (HD) is a debilitating, currently incurable disease. Protein aggregation
and metabolic deficits are pathological hallmarks but their link to neurodegeneration and …

Enhancers and chromatin structures: regulatory hubs in gene expression and diseases

Z Hu, WW Tee - Bioscience reports, 2017 - portlandpress.com
Gene expression requires successful communication between enhancer and promoter
regions, whose activities are regulated by a variety of factors and associated with distinct …

Super enhancers in cancers, complex disease, and developmental disorders

AR Niederriter, A Varshney, SCJ Parker, DM Martin - Genes, 2015 - mdpi.com
Recently, unique areas of transcriptional regulation termed super-enhancers have been
identified and implicated in human disease. Defined by their magnitude of size, transcription …

Huntington's disease-specific mis-splicing unveils key effector genes and altered splicing factors

A Elorza, Y Márquez, JR Cabrera… - Brain, 2021 - academic.oup.com
Correction of mis-splicing events is a growing therapeutic approach for neurological
diseases such as spinal muscular atrophy or neuronal ceroid lipofuscinosis 7, which are …