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Small, Seeding-Competent Huntingtin Fibrils Are Prominent Aggregate Species in Brains of zQ175 Huntington’s Disease Knock-in Mice

Autor: Schindler, Franziska, Praedel, Nicole, Neuendorf, Nancy, Kunz, Severine, Schnoegl, Sigrid, Mason, Michael A., Taxy, Bridget A., Bates, Gillian P., Khoshnan, Ali, Priller, Josef, Grimm, Jan, Maier, Marcel, Boeddrich, Annett, Wanker, Erich E.

Publikováno v: Frontiers in Neuroscience
Frontiers in neuroscience 15, 682172 (2021). doi:10.3389/fnins.2021.682172

The deposition of mutant huntingtin (mHTT) protein aggregates in neurons of patients is a pathological hallmark of Huntington’s disease (HD). Previous investigations in cell-free and cell-based disease models showed mHTT exon-1 (mHTTex1) fragments

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::bd29dcfcc5714c9af61e2c80d82a9915

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Development of novel bioassays to detect soluble and aggregated Huntingtin proteins on three technology platforms.

Autor: Landles C; Department of Neurodegenerative Disease, Huntington's Disease Centre, UK Dementia Research Institute at UCL, Queen Square Institute of Neurology, University College London, London, UK., Milton RE; Department of Neurodegenerative Disease, Huntington's Disease Centre, UK Dementia Research Institute at UCL, Queen Square Institute of Neurology, University College London, London, UK., Jean A; Perkin Elmer Inc., Seer Green, UK., McLarnon S; Perkin Elmer Inc., Seer Green, UK., McAteer SJ; Department of Neurodegenerative Disease, Huntington's Disease Centre, UK Dementia Research Institute at UCL, Queen Square Institute of Neurology, University College London, London, UK., Taxy BA; Department of Neurodegenerative Disease, Huntington's Disease Centre, UK Dementia Research Institute at UCL, Queen Square Institute of Neurology, University College London, London, UK., Osborne GF; Department of Neurodegenerative Disease, Huntington's Disease Centre, UK Dementia Research Institute at UCL, Queen Square Institute of Neurology, University College London, London, UK., Zhang C; Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA.; Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Duan W; Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, USA.; Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Howland D; CHDI Management/CHDI Foundation Inc., New York, NY, USA., Bates GP; Department of Neurodegenerative Disease, Huntington's Disease Centre, UK Dementia Research Institute at UCL, Queen Square Institute of Neurology, University College London, London, UK.

Publikováno v: Brain communications [Brain Commun] 2021 Jan 05; Vol. 3 (1), pp. fcaa231. Date of Electronic Publication: 2021 Jan 05 (Print Publication: 2021).