Category Archives: Huntington

Chaperone-Dependent Mechanisms as a Pharmacological Target for Neuroprotection

Chaperone-Dependent Mechanisms as a Pharmacological Target for Neuroprotection

By Mikhail V. Voronin, Elena V. Abramova, Ekaterina R. Verbovaya, Yulia V. Vakhitova, and Sergei B. Seredenin Excerpt from the article published in International Journal of Molecular Sciences 24, no. 1: 823. 3 January 2023, DOI: https://doi.org/10.3390/ijms24010823 Editor’s Highlights Abstract Modern pharmacotherapy of neurodegenerative diseases is predominantly symptomatic and does not allow vicious circles causing disease […]

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Mitochondria in Huntington’s disease: implications in pathogenesis and mitochondrial-targeted therapeutic strategies

Mitochondria in Huntington’s disease: implications in pathogenesis and mitochondrial-targeted therapeutic strategies

By Ana Maria Jurcau, and Carolina Maria Jurcau Excerpt from the article published in Neural Regeneration Research 18(7):p 1472-1477, July 2023. | DOI: 10.4103/1673-5374.360289 Editor’s Highlights Abstract Huntington’s disease is a genetic disease caused by expanded CAG repeats on exon 1 of the huntingtin gene located on chromosome 4. Compelling evidence implicates impaired mitochondrial energetics, […]

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Huntington disease oligodendrocyte maturation deficits revealed by single-nucleus RNAseq are rescued by thiamine-biotin supplementation

Huntington disease oligodendrocyte maturation deficits revealed by single-nucleus RNAseq are rescued by thiamine-biotin supplementation

By Ryan G. Lim, Osama Al-Dalahmah, Jie Wu, Maxwell P. Gold, Jack C. Reidling, Guomei Tang, Miriam Adam, David K. Dansu, Hye-Jin Park, Patrizia Casaccia, Ricardo Miramontes, Andrea M. Reyes-Ortiz, Alice Lau, Richard A. Hickman, Fatima Khan, Fahad Paryani, Alice Tang, Kenneth Ofori, Emily Miyoshi, Neethu Michael, Nicolette McClure, Xena E. Flowers, Jean Paul Vonsattel, […]

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A Novel Huntington’s Disease Assessment Platform to Support Future Drug Discovery and Development

A Novel Huntington’s Disease Assessment Platform to Support Future Drug Discovery and Development

By Jingyun Wu, Luisa Möhle, Thomas Brüning Iván Eiriz, Muhammad Rafehi, Katja Stefan, Sven Marcel Stefan, and Jens Pahnke Excerpt from the article published in International Journal of Molecular Sciences 23, no. 23: 14763, 25 November 2022. DOI: https://doi.org/10.3390/ijms232314763 Editor’s Highlights Abstract Huntington’s disease (HD) is a lethal neurodegenerative disorder without efficient therapeutic options. The inefficient translation […]

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Targeting Molecular Mediators of Ferroptosis and Oxidative Stress for Neurological Disorders

Targeting Molecular Mediators of Ferroptosis and Oxidative Stress for Neurological Disorders

By Jing Li, Bowen Jia, Ying Cheng, Yiting Song, Qianqian Li, and Chengliang Luo Excerpt from the article published in Oxidative Medicine and Cellular Longevity, Volume 2022, Article ID 3999083, Published 22 Jul 2022, DOI: https://doi.org/10.1155/2022/3999083 Editor’s Highlights Abstract As a novel mode of regulated cell death (RCD), ferroptosis was firstly proposed by Dixon in 2012 [1]. With […]

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Alterations in metal homeostasis occur prior to canonical markers in Huntington disease

Alterations in metal homeostasis occur prior to canonical markers in Huntington disease

By Anna C. Pfalzer, Yan Yan, Hakmook Kang, Melissa Totten, James Silverman, Aaron B. Bowman, Keith Erikson, and Daniel O. Claassen Excerpt from the article published in Scientific Reports 12, 10373, 20 June 2022, DOI: https://doi.org/10.1038/s41598-022-14169-y Editor’s Highlights Essential trace metals play a vital role in several metabolic processes throughout the body and brain. The […]

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Neuroprotective Effects of σ2R/TMEM97 Receptor Modulators in Neuronal Model of Huntington’s Disease

Neuroprotective Effects of σ2R/TMEM97 Receptor Modulators in Neuronal Model of Huntington’s Disease

By Jing Jin, Nicolas Arbez, James J. Sahn, Yan Lu, Kathryn T. Linkens, Timothy R. Hodges, Anthony Tang, Robyn Wiseman, Stephen F. Martin, and Christopher A. Ross Excerpt from the preprint published by ChemRxiv. Cambridge: Cambridge Open Engage; 2022; DOI 10.26434/chemrxiv-2022-l6xtv This content has not been peer-reviewed. Editor’s Highlights Huntington’s disease (HD) is a genetic […]

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Analysis of LINE1 Retrotransposons in Huntington’s Disease

Analysis of LINE1 Retrotransposons in Huntington’s Disease

By Lavinia Floreani, Federico Ansaloni, Damiano Mangoni, Elena Agostoni, Remo Sanges, Francesca Persichetti and Stefano Gustincich Excerpt from the article published in Frontiers in Cellular Neuroscience, 14 January 2022 | https://doi.org/10.3389/fncel.2021.743797 Editor’s Highlights Abstract Transposable elements (TEs) are mobile genetic elements that made up about half the human genome. Among them, the autonomous non-LTR retrotransposon long […]

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Nuclear and cytoplasmic huntingtin inclusions exhibit distinct biochemical composition, interactome and ultrastructural properties

Nuclear and cytoplasmic huntingtin inclusions exhibit distinct biochemical composition, interactome and ultrastructural properties

By Nathan Riguet, Anne-Laure Mahul-Mellier, Niran Maharjan, Johannes Burtscher, Marie Croisier, Graham Knott, Janna Hastings, Alice Patin, Veronika Reiterer, Hesso Farhan, Sergey Nasarov, and Hial A. Lashuel Excerpt from the article published in Nature Communications, volume 12, Article number: 6579 (2021) DOI: https://doi.org/10.1038/s41467-021-26684-z Editor’s Highlights Huntington’s disease (HD) is an autosomal dominant genetic and progressive neurodegenerative disorder caused by the abnormal […]

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Juvenile-Onset Huntington Disease Pathophysiology and Neurodevelopment: A Review

Juvenile-Onset Huntington Disease Pathophysiology and Neurodevelopment: A Review

By Hannah S. Bakels, MSc, Raymund A.C. Roos, Willeke M.C. van Roon-Mom, and Susanne T. de Bot Excerpt from the article published online 12 October 2021 in Wiley Online Library(wileyonlinelibrary.com). DOI: 10.1002/mds.28823 Editor’s Highlights Huntington disease (HD) is an autosomal dominant progressive brain disorder caused by a pathological CAG repeat expansion coding for huntingtin (HTT gene), […]

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