Activating transcription factor 6 derepression mediates neuroprotection in Huntington disease

JR Naranjo, H Zhang, D Villar… - The Journal of …, 2016 - Am Soc Clin Investig
Deregulated protein and Ca2+ homeostasis underlie synaptic dysfunction and
neurodegeneration in Huntington disease (HD); however, the factors that disrupt …

Inhibition of DREAM-ATF6 interaction delays onset of cognition deficit in a mouse model of Huntington's disease

A López-Hurtado, DF Burgos, P González, XM Dopazo… - Molecular Brain, 2018 - Springer
The transcriptional repressor DREAM (downstream regulatory element antagonist
modulator) is a multifunctional neuronal calcium sensor (NCS) that controls Ca 2+ and …

Targeting the neuronal calcium sensor DREAM with small-molecules for Huntington's disease treatment

A Lopez-Hurtado, DA Peraza, P Cercos, L Lagartera… - Scientific reports, 2019 - nature.com
DREAM, a neuronal calcium sensor protein, has multiple cellular roles including the
regulation of Ca2+ and protein homeostasis. We recently showed that reduced DREAM …

The neuroprotective transcription factor ATF5 is decreased and sequestered into polyglutamine inclusions in Huntington's disease

IH Hernández, J Torres-Peraza, M Santos-Galindo… - Acta …, 2017 - Springer
Abstract Activating transcription factor-5 (ATF5) is a stress-response transcription factor
induced upon different cell stressors like fasting, amino-acid limitation, cadmium or arsenite …

Targeting TEAD/YAP-transcription-dependent necrosis, TRIAD, ameliorates Huntington's disease pathology

Y Mao, X Chen, M Xu, K Fujita, K Motoki… - Human molecular …, 2016 - academic.oup.com
Neuronal cell death in neurodegenerative diseases is not fully understood. Here we report
that mutant huntingtin (Htt), a causative gene product of Huntington's diseases (HD) …

Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets

M Jiang, J Wang, J Fu, L Du, H Jeong, T West… - Nature medicine, 2012 - nature.com
Huntington's disease is a fatal neurodegenerative disorder caused by an expanded
polyglutamine repeat in huntingtin (HTT) protein. We previously showed that calorie …

[HTML][HTML] Neuroprotection for Huntington's disease: ready, set, slow

SM Hersch, HD Rosas - Neurotherapeutics, 2008 - Elsevier
The ultimate goal for Huntington's disease (HD) therapeutics is to develop disease-
modifying neuroprotective therapies that can delay or prevent illness in those who are at …

Mutant huntingtin activates Nrf2-responsive genes and impairs dopamine synthesis in a PC12 model of Huntington's disease

WMC van Roon-Mom, BA Pepers, PAC 't Hoen… - BMC Molecular …, 2008 - Springer
Background Huntington's disease is a progressive autosomal dominant neurodegenerative
disorder that is caused by a CAG repeat expansion in the HD or Huntington's disease gene …

Golgi stress response reprograms cysteine metabolism to confer cytoprotection in Huntington's disease

JI Sbodio, SH Snyder, BD Paul - Proceedings of the …, 2018 - National Acad Sciences
Golgi stress response is emerging as a physiologic process of comparable importance to
endoplasmic reticulum (ER) and mitochondrial stress responses. However, unlike ER stress …

Huntington's disease and its therapeutic target genes: a global functional profile based on the HD Research Crossroads database

RKR Kalathur, MA Hernández-Prieto, ME Futschik - BMC neurology, 2012 - Springer
Background Huntington's disease (HD) is a fatal progressive neurodegenerative disorder
caused by the expansion of the polyglutamine repeat region in the huntingtin gene …