دورية أكاديمية
Development of mAb-based polyglutamine-dependent and polyglutamine length-independent huntingtin quantification assays with cross-site validation.
| العنوان: | Development of mAb-based polyglutamine-dependent and polyglutamine length-independent huntingtin quantification assays with cross-site validation. |
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| المؤلفون: | David F Fischer, Sipke Dijkstra, Kimberly Lo, Johnny Suijker, Ana C P Correia, Patricia Naud, Martin Poirier, Michela A Tessari, Ivette Boogaard, Geraldine Flynn, Mijke Visser, Marieke B A C Lamers, George McAllister, Ignacio Munoz-Sanjuan, Douglas Macdonald |
| المصدر: | PLoS ONE, Vol 17, Iss 4, p e0266812 (2022) |
| بيانات النشر: | Public Library of Science (PLoS) |
| سنة النشر: | 2022 |
| المجموعة: | Directory of Open Access Journals: DOAJ Articles |
| مصطلحات موضوعية: | Medicine, Science |
| الوصف: | Huntington's disease (HD) is caused by an expansion of the CAG trinucleotide repeat domain in the huntingtin gene that results in expression of a mutant huntingtin protein (mHTT) containing an expanded polyglutamine tract in the amino terminus. A number of therapeutic approaches that aim to reduce mHTT expression either locally in the CNS or systemically are in clinical development. We have previously described sensitive and selective assays that measure human HTT proteins either in a polyglutamine-independent (detecting both mutant expanded and non-expanded proteins) or in a polyglutamine length-dependent manner (detecting the disease-causing polyglutamine repeats) on the electrochemiluminescence Meso Scale Discovery detection platform. These original assays relied upon polyclonal antibodies. To ensure an accessible and sustainable resource for the HD field, we developed similar assays employing monoclonal antibodies. We demonstrate that these assays have equivalent sensitivity compared to our previous assays through the evaluation of cellular and animal model systems, as well as HD patient biosamples. We also demonstrate cross-site validation of these assays, allowing direct comparison of studies performed in geographically distinct laboratories. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| تدمد: | 1932-6203 |
| العلاقة: | https://doi.org/10.1371/journal.pone.0266812Test; https://doaj.org/toc/1932-6203Test; https://doaj.org/article/d40329dcbdbd4ce6b214f506ecdfe85cTest |
| DOI: | 10.1371/journal.pone.0266812 |
| الإتاحة: | https://doi.org/10.1371/journal.pone.0266812Test https://doaj.org/article/d40329dcbdbd4ce6b214f506ecdfe85cTest |
| رقم الانضمام: | edsbas.A572377F |
| قاعدة البيانات: | BASE |
Full Text Finder | تدمد: | 19326203 |
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| DOI: | 10.1371/journal.pone.0266812 |