التفاصيل البيبلوغرافية
| العنوان: |
Contingent intramuscular boosting of P2XR7 axis improves motor function in transgenic ALS mice |
| المؤلفون: |
Fabbrizio, Paola, D’Agostino, Jessica, Margotta, Cassandra, Mella, Giulia, Panini, Nicolò, Pasetto, Laura, Sammali, Eliana, Raggi, Flavia, Sorarù, Gianni, Bonetto, Valentina, Bendotti, Caterina, Nardo, Giovanni |
| المساهمون: |
Ministero della Salute, Fondazione Italiana di Ricerca per la Sclerosi Laterale Amiotrofica, Regione Lombardia |
| المصدر: |
Cellular and Molecular Life Sciences ; volume 79, issue 1 ; ISSN 1420-682X 1420-9071 |
| بيانات النشر: |
Springer Science and Business Media LLC |
| سنة النشر: |
2021 |
| مصطلحات موضوعية: |
Cell Biology, Cellular and Molecular Neuroscience, Pharmacology, Molecular Biology, Molecular Medicine |
| الوصف: |
Amyotrophic lateral sclerosis is a fatal neurodegenerative disorder that leads to progressive degeneration of motor neurons and severe muscle atrophy without effective treatment. Most research on the disease has been focused on studying motor neurons and supporting cells of the central nervous system. Strikingly, the recent observations have suggested that morpho-functional alterations in skeletal muscle precede motor neuron degeneration, bolstering the interest in studying muscle tissue as a potential target for the delivery of therapies. We previously showed that the systemic administration of the P2XR7 agonist, 2′(3′)- O ‐(4-benzoylbenzoyl) adenosine 5-triphosphate (BzATP), enhanced the metabolism and promoted the myogenesis of new fibres in the skeletal muscles of SOD1G93A mice. Here we further corroborated this evidence showing that intramuscular administration of BzATP improved the motor performance of ALS mice by enhancing satellite cells and the muscle pro-regenerative activity of infiltrating macrophages. The preservation of the skeletal muscle retrogradely propagated along with the motor unit, suggesting that backward signalling from the muscle could impinge on motor neuron death. In addition to providing the basis for a suitable adjunct multisystem therapeutic approach in ALS, these data point out that the muscle should be at the centre of ALS research as a target tissue to address novel therapies in combination with those oriented to the CNS. |
| نوع الوثيقة: |
article in journal/newspaper |
| اللغة: |
English |
| DOI: |
10.1007/s00018-021-04070-8 |
| DOI: |
10.1007/s00018-021-04070-8.pdf |
| DOI: |
10.1007/s00018-021-04070-8/fulltext.html |
| الإتاحة: |
https://doi.org/10.1007/s00018-021-04070-8Test |
| حقوق: |
https://creativecommons.org/licenses/by/4.0Test ; https://creativecommons.org/licenses/by/4.0Test |
| رقم الانضمام: |
edsbas.BAC7E6C0 |
| قاعدة البيانات: |
BASE |
