Results 31 to 40 of about 19,992 (185)

Gene Silencing Therapies for Huntington’s Disease [PDF]

Neurology Letters
Huntington’s disease (HD) is a rare, autosomal‑dominant neurodegenerative disorder precipitated by a pathological expansion of CAG trinucleotide repeats in exon 1 of the huntingtin (HTT) gene.
Chunlan Hao, Hanfeng Ji
doaj   +1 more source

The localization and interactions of huntingtin [PDF]

Philosophical Transactions of the Royal Society of London. Series B: Biological Sciences, 1999
Huntingtin was localized by using a series of antibodies that detected different areas of the protein from the immediate N–terminus to the C–terminal region of the protein. The more C–terminal antibodies gave a cytoplasmic localization in neurons of the brain in controls and cases of Huntington'sdisease (HD).
openaire   +2 more sources

Studying Huntington’s Disease in Yeast: From Mechanisms to Pharmacological Approaches

Frontiers in Molecular Neuroscience, 2018
Huntington’s disease (HD) is a neurodegenerative disorder that leads to progressive neuronal loss, provoking impaired motor control, cognitive decline, and dementia.
Sebastian Hofer, Katharina Kainz, Andreas Zimmermann, Andreas Zimmermann, Maria A. Bauer, Tobias Pendl, Michael Poglitsch, Frank Madeo, Frank Madeo, Didac Carmona-Gutierrez   +9 more
doaj   +1 more source

Altering cortical input unmasks synaptic phenotypes in the YAC128 cortico-striatal co-culture model of Huntington disease

BMC Biology, 2018
Background Huntington disease (HD) is a fatal neurodegenerative disorder caused by a CAG expansion in the huntingtin (HTT) gene, leading to selective and progressive neuronal death predominantly in the striatum. Mutant HTT expression causes dysfunctional
Mandi E. Schmidt, Caodu Buren, James P. Mackay, Daphne Cheung, Louisa Dal Cengio, Lynn A. Raymond, Michael R. Hayden   +6 more
doaj   +1 more source

PolyQ-Expansion Causes Mitochondria Fragmentation Independent of Huntingtin and Is Distinct from Traumatic Brain Injury (TBI)/Mechanical Stress-Mediated Fragmentation Which Results from Cell Death

Cells, 2023
Mitochondrial dysfunction has been reported in many Huntington’s disease (HD) models; however, it is unclear how these defects occur. Here, we test the hypothesis that excess pathogenic huntingtin (HTT) impairs mitochondrial homeostasis, using Drosophila
Kelsey Swinter, Dania Salah, Rasika Rathnayake, Shermali Gunawardena   +3 more
doaj   +1 more source

Safe and Efficient Silencing with a Pol II, but Not a Pol lII, Promoter Expressing an Artificial miRNA Targeting Human Huntingtin

Molecular Therapy: Nucleic Acids, 2017
Huntington’s disease is a devastating, incurable neurodegenerative disease affecting up to 12 per 100,000 patients worldwide. The disease is caused by a mutation in the Huntingtin (Htt) gene.
Edith L. Pfister, Kathryn O. Chase, Huaming Sun, Lori A. Kennington, Faith Conroy, Emily Johnson, Rachael Miller, Florie Borel, Neil Aronin, Christian Mueller   +9 more
doaj   +1 more source

Huntingtin-mediated axonal transport requires arginine methylation by PRMT6

Cell Reports, 2021
Summary: The huntingtin (HTT) protein transports various organelles, including vesicles containing neurotrophic factors, from embryonic development throughout life. To better understand how HTT mediates axonal transport and why this function is disrupted
Alice Migazzi, Chiara Scaramuzzino, Eric N. Anderson, Debasmita Tripathy, Ivó H. Hernández, Rogan A. Grant, Michela Roccuzzo, Laura Tosatto, Amandine Virlogeux, Chiara Zuccato, Andrea Caricasole, Tamara Ratovitski, Christopher A. Ross, Udai B. Pandey, José J. Lucas, Frédéric Saudou, Maria Pennuto, Manuela Basso   +17 more
doaj   +1 more source

Hsp40 overexpression in pacemaker neurons delays circadian dysfunction in a Drosophila model of Huntington's disease

Disease Models & Mechanisms, 2022
Circadian disturbances are early features of neurodegenerative diseases, including Huntington's disease (HD). Emerging evidence suggests that circadian decline feeds into neurodegenerative symptoms, exacerbating them.
Pavitra Prakash, Arpit Kumar Pradhan, Vasu Sheeba   +2 more
doaj   +1 more source

Introducing Borsantrazole: A Trifunctional Boron‐Based Pyrazole That Extends the Lifespan of Amyotrophic Lateral Sclerosis Mice

Advanced Science, EarlyView.
Herein we report a boron‐based pyrazole, (Borsantrazole ‐ a small molecule that selectively targets oxidative stress) that significantly increases survival, reduces weight loss, delays disease onset, and affects global protein changes in the SOD1‐G37R mouse model of ALS.
Nitesh Sanghai, Rhonda Kelley, Ying Lao, M. Immanuel Reyes Madlangsakay, Prasanta Paul, M. Alejandra Llanes‐Cuesta, Jun‐Feng Wang, René P. Zahedi, Jiming Kong, Geoffrey K. Tranmer   +9 more
wiley   +1 more source

Structural Polymorphism of polyG Inclusions Revealed by In Situ Cryo‐Electron Tomography

Advanced Science, EarlyView.
Correlative cryo‐electron tomography in primary cortical neurons and NIID mouse brain tissue reveals that polyG inclusions are interconnected ribbon‐like assemblies rather than canonical amyloid fibrils. Multiple compartment‐specific ribbon states show distinct 26S proteasome accessibility, while cytoplasmic ribbons contact and deform ER‐like ...
Yunwen Qian, Yalan Wan, Chen Chen, Xiaoyu Zheng, Wenjing Du, Junhan Yang, Zhihao Quan, Biyu Yang, Jiaxi Yu, Jie Zheng, Zhaoxia Wang, Jianwen Deng, Qiang Guo   +12 more
wiley   +1 more source