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Fabbrizio, P, Baindoor, S, Margotta, C, Su, J, Morrissey, EP, Woods, I, Hogg, MC 
ORCID: https://orcid.org/0000-0002-2815-8761, Vianello, S, Venø, MT, Kjems, J, Sorarù, G, Bendotti, C, Prehn, JHM and Nardo, G,
2024.
Protective role of angiogenin in muscle regeneration in amyotrophic lateral sclerosis: diagnostic and therapeutic implications.
Brain Pathology: e13328.
ISSN 1015-6305
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Abstract
Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disease with no effective treatments, in part caused by variations in progression and the absence of biomarkers. Mice carrying the SOD1G93A transgene with different genetic backgrounds show variable disease rates, reflecting the diversity of patients. While extensive research has been done on the involvement of the central nervous system, the role of skeletal muscle remains underexplored. We examined the impact of angiogenin, including its RNase activity, in skeletal muscles of ALS mouse models and in biopsies from ALS patients. Elevated levels of angiogenin were found in slowly progressing mice but not in rapidly progressing mice, correlating with increased muscle regeneration and vascularisation. In patients, higher levels of angiogenin in skeletal muscles correlated with milder disease. Mechanistically, angiogenin promotes muscle regeneration and vascularisation through satellite cell-endothelial interactions during myogenesis and angiogenesis. Furthermore, specific angiogenin-derived tiRNAs were upregulated in slowly progressing mice, suggesting their role in mediating the effects of angiogenin. These findings highlight angiogenin and its tiRNAs as potential prognostic markers and therapeutic targets for ALS, offering avenues for patient stratification and interventions to mitigate disease progression by promoting muscle regeneration.
| Item Type: | Journal article |
|---|---|
| Publication Title: | Brain Pathology |
| Creators: | Fabbrizio, P., Baindoor, S., Margotta, C., Su, J., Morrissey, E.P., Woods, I., Hogg, M.C., Vianello, S., Venø, M.T., Kjems, J., Sorarù, G., Bendotti, C., Prehn, J.H.M. and Nardo, G. |
| Publisher: | Wiley |
| Date: | 28 December 2024 |
| ISSN: | 1015-6305 |
| Identifiers: | Number Type 10.1111/bpa.13328 DOI 2332350 Other |
| Rights: | © 2024 the author(s). Brain Pathology published by John Wiley & Sons Ltd on behalf of International Society of Neuropathology. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made. |
| Record created by: | Jonathan Gallacher |
| Date Added: | 07 Jan 2025 15:15 |
| Last Modified: | 07 Jan 2025 15:15 |
| URI: | https://irep.ntu.ac.uk/id/eprint/52796 |
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