دورية أكاديمية
CD8+ T cell depletion prevents neuropathology in a mouse model of globoid cell leukodystrophy.
| العنوان: | CD8+ T cell depletion prevents neuropathology in a mouse model of globoid cell leukodystrophy. |
|---|---|
| المؤلفون: | Sutter, Pearl A, Ménoret, Antoine, Jellison, Evan R, Nicaise, Alexandra M, Bradbury, Allison M, Vella, Anthony T, Bongarzone, Ernesto R, Crocker, Stephen J |
| المصدر: | nlmid: 2985109R ; essn: 1540-9538 |
| بيانات النشر: | Rockefeller University Press //dx.doi.org/10.1084/jem.20221862 J Exp Med |
| سنة النشر: | 2023 |
| المجموعة: | Apollo - University of Cambridge Repository |
| مصطلحات موضوعية: | Animals, Mice, Leukodystrophy, Globoid Cell, Central Nervous System, Disease Models, Animal, Antibodies, Blocking, CD8-Positive T-Lymphocytes |
| الوصف: | Globoid cell leukodystrophy (GLD) or Krabbe's disease is a fatal genetic demyelinating disease of the central nervous system caused by loss-of-function mutations in the galactosylceramidase (galc) gene. While the metabolic basis for disease is known, the understanding of how this results in neuropathology is not well understood. Herein, we report that the rapid and protracted elevation of CD8+ cytotoxic T lymphocytes occurs coincident with clinical disease in a mouse model of GLD. Administration of a function-blocking antibody against CD8α effectively prevented disease onset, reduced morbidity and mortality, and prevented CNS demyelination in mice. These data indicate that subsequent to the genetic cause of disease, neuropathology is driven by pathogenic CD8+ T cells, thus offering novel therapeutic potential for treatment of GLD. |
| نوع الوثيقة: | article in journal/newspaper |
| وصف الملف: | application/pdf |
| اللغة: | English |
| العلاقة: | https://www.repository.cam.ac.uk/handle/1810/354056Test |
| الإتاحة: | https://www.repository.cam.ac.uk/handle/1810/354056Test |
| حقوق: | Attribution 4.0 International ; https://creativecommons.org/licenses/by/4.0Test/ |
| رقم الانضمام: | edsbas.8AFF03E |
| قاعدة البيانات: | BASE |
| الوصف غير متاح. |