Academic Journal
Knockdown of Muscle-Specific Ribosomal Protein L3-Like Enhances Muscle Function in Healthy and Dystrophic Mice.
| Title: | Knockdown of Muscle-Specific Ribosomal Protein L3-Like Enhances Muscle Function in Healthy and Dystrophic Mice. |
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| Authors: | Kao BR; Department of Biological Sciences, School of Life Sciences and the Environment, Royal Holloway University of London, Egham, United Kingdom., Malerba A; Department of Biological Sciences, School of Life Sciences and the Environment, Royal Holloway University of London, Egham, United Kingdom., Lu-Nguyen NB; Department of Biological Sciences, School of Life Sciences and the Environment, Royal Holloway University of London, Egham, United Kingdom., Harish P; Department of Biological Sciences, School of Life Sciences and the Environment, Royal Holloway University of London, Egham, United Kingdom., McCarthy JJ; Department of Physiology, University of Kentucky, Lexington, Kentucky, USA., Dickson G; Department of Biological Sciences, School of Life Sciences and the Environment, Royal Holloway University of London, Egham, United Kingdom., Popplewell LJ; Department of Biological Sciences, School of Life Sciences and the Environment, Royal Holloway University of London, Egham, United Kingdom. |
| Source: | Nucleic acid therapeutics [Nucleic Acid Ther] 2021 Dec; Vol. 31 (6), pp. 457-464. Date of Electronic Publication: 2021 Jun 02. |
| Publication Type: | Journal Article; Research Support, Non-U.S. Gov't |
| Language: | English |
| Journal Info: | Publisher: Mary Ann Liebert, Inc Country of Publication: United States NLM ID: 101562758 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2159-3345 (Electronic) Linking ISSN: 21593337 NLM ISO Abbreviation: Nucleic Acid Ther Subsets: MEDLINE |
| Imprint Name(s): | Original Publication: New Rochelle, N.Y. : Mary Ann Liebert, Inc. |
| MeSH Terms: | Muscular Dystrophy, Duchenne*/genetics , Muscular Dystrophy, Duchenne*/therapy , Ribosomal Protein L3*, Animals ; Disease Models, Animal ; Dystrophin ; Mice ; Mice, Inbred mdx ; Muscle Contraction ; Muscle, Skeletal |
| Abstract: | Ribosomal protein L3-like (RPL3L) is a poorly characterized ribosomal protein that is exclusively expressed in skeletal and cardiac muscle. RPL3L is also downregulated in Duchenne muscular dystrophy (DMD), suggesting that it may play an important role in muscle biology. In this study, we investigated the role of RPL3L in skeletal muscle of healthy C57 and dystrophic mdx mice. We show that RPL3L is developmentally regulated and that intramuscular adeno-associated virus (AAV)-mediated RPL3L knockdown in the tibialis anterior of C57 and mdx mice results in increased specific force with improved resistance to eccentric contraction induced muscle damage in dystrophic muscles. The mechanism by which RPL3L knockdown improves muscle function remains unclear. Histological observations showed a significant increase in muscle length and decrease in muscle cross-sectional area after RPL3L inhibition suggesting that this ribosomal protein may play a role in myofiber morphology. The endogenous downregulation of RPL3L in DMD may be a protective mechanism that attempts to improve skeletal muscle function and counteract the dystrophic phenotype. |
| Contributed Indexing: | Keywords: Duchenne muscular dystrophy; gene therapy; in vivo muscle electrophysiology; muscle function; ribosomal protein |
| Substance Nomenclature: | 0 (Dystrophin) 0 (Ribosomal Protein L3) |
| Entry Date(s): | Date Created: 20210603 Date Completed: 20220113 Latest Revision: 20220531 |
| Update Code: | 20260130 |
| DOI: | 10.1089/nat.2020.0928 |
| PMID: | 34081545 |
| Database: | MEDLINE |
| ISSN: | 2159-3345 |
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| DOI: | 10.1089/nat.2020.0928 |