Article
Plant Sciences
Inae Sim, Jaewoong Jang, Jaewon Song, Jongkyu Lee, Hyemi Lim, Hyun Jung Lee, Gyusik Hwang, Young V. Kwon, Doheon Lee, Yoosik Yoon
Summary: Paeonia lactiflora Pall. extract improves muscle function and reduces muscle damage in mdx mice by suppressing HMGB1-TLR4-NF-kappa B signaling and downregulating proinflammatory cytokines/chemokines.
JOURNAL OF ETHNOPHARMACOLOGY
(2022)
Review
Cell Biology
Elisa Domi, Malvina Hoxha, Emanuela Prendi, Bruno Zappacosta
Summary: Duchenne muscular dystrophy is a muscular disease with no cure, and SIRT1 has been identified as a potential therapeutic target for the condition. Activation of SIRT1 improves muscle function, while its inhibition leads to muscle fragility.
Article
Pharmacology & Pharmacy
Shagun Singh, Tejpal Singh, Chaitanya Kunja, Navdeep S. Dhoat, Narender K. Dhania
Summary: Muscular dystrophy is a genetically heterogeneous rare muscle disorder that leads to muscle breakdown and weakness, with therapeutics including antioxidants, anti-inflammatory drugs, and induced pluripotent stem cells for regeneration.
EUROPEAN JOURNAL OF PHARMACOLOGY
(2021)
Article
Medicine, Research & Experimental
Cedric Happi Mbakam, Joel Rousseau, Yaoyao Lu, Anne Bigot, Kamel Mamchaoui, Vincent Mouly, Jacques P. Tremblay
Summary: In this study, researchers used CRISPR-Cas9 prime editing technology to correct a mutation in the DMD gene, resulting in improved editing efficiency and restoration of dystrophin protein expression. Optimization of the reverse transcription template sequence led to a significant increase in the editing percentage of the target nucleotide.
MOLECULAR THERAPY-NUCLEIC ACIDS
(2022)
Review
Biochemistry & Molecular Biology
Luana Tripodi, Chiara Villa, Davide Molinaro, Yvan Torrente, Andrea Farini
Summary: The interaction between the immune system and skeletal muscle plays a crucial role in inflammatory muscle diseases and dystrophic conditions, affecting muscle regeneration and pathogenesis.
Article
Clinical Neurology
Richard S. Finkel, Craig M. McDonald, H. Lee Sweeney, Erika Finanger, Erin Neil Knierbein, Kathryn R. Wagner, Katherine D. Mathews, Warren Marks, Jeffrey Statland, Jessica Nance, Hugh J. McMillan, Gary McCullagh, Cuixia Tian, Monique M. Ryan, Declan O'Rourke, Wolfgang Mueller-Felber, Mar Tulinius, W. Bryan Burnette, Cam-Tu Nguyen, Kayal Vijayakumar, Jessika Johannsen, Han C. Phan, Michelle Eagle, James MacDougall, Maria Mancini, Joanne M. Donovan
Summary: Edasalonexent did not show significant improvement in muscle function in DMD patients, but may slow disease progression in younger patients. Most patients tolerated the treatment well with mild adverse events primarily involving the gastrointestinal system.
JOURNAL OF NEUROMUSCULAR DISEASES
(2021)
Article
Biochemistry & Molecular Biology
Alexander B. Andre, Liqiang Zhang, Jalen D. Nix, Nora Elmadbouly, Alexandra R. Lucas, Jeanne Wilson-Rawls, Alan Rawls
Summary: Duchenne muscular dystrophy is a rare disease that affects males and current treatment options have serious side effects. A study suggests that a new immune-modulating protein called Pegylated Serp-1 can improve inflammation and fibrosis, potentially providing a new therapeutic approach for Duchenne muscular dystrophy.
Article
Biochemistry & Molecular Biology
Maura Argenziano, Vincenzo Pota, Alessandra Di Paola, Chiara Tortora, Maria Maddalena Marrapodi, Giulia Giliberti, Domenico Roberti, Maria Caterina Pace, Francesca Rossi
Summary: Duchenne Muscular Dystrophy (DMD) is a severe disease caused by a mutation in the DMD gene, leading to muscular degeneration and secondary complications. The main therapy for DMD is corticosteroids, but the side effects call for safer alternatives. Macrophages, immune cells expressing the CB2 receptor, have been suggested as a target for anti-inflammatory treatment. In this study, the CB2 receptor agonist JWH-133 was found to have a beneficial effect by reducing pro-inflammatory cytokines and promoting anti-inflammatory macrophage phenotype in DMD-associated macrophages.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Biochemistry & Molecular Biology
E. Panza, V. Vellecco, F. A. Iannotti, D. Paris, O. L. Manzo, M. Smimmo, N. Mitilini, A. Boscaino, G. de Dominicis, M. Bucci, A. Di Lorenzo, G. Cirino
Summary: The study reveals a defective TSP pathway activity in DMD and demonstrates that H2S donors can effectively recover impaired locomotor activity and reduce inflammation in skeletal muscle tissues of both DMD patients and mdx mice.
Article
Biochemistry & Molecular Biology
Zsofia Onodi, Petra Lujza Szabo, Daniel Kucsera, Peter Pokreisz, Christopher Dostal, Karlheinz Hilber, Gavin Y. Oudit, Bruno K. Podesser, Peter Ferdinandy, Zoltan V. Varga, Attila Kiss
Summary: Duchenne muscular dystrophy (DMD) is a muscle wasting disease characterized by difficulty moving and premature death, mainly due to heart failure. Inflammation is thought to play a role in the disease progression, but the specific mechanisms are not well understood.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Clinical Neurology
Giulio Gadaleta, Guido Urbano, Chiara Brusa, Rossella D'Alessandro, Enrica Rolle, Ilaria Cavallina, Alessio Mattei, Fulvia Ribolla, Claudia Raineri, Stefano Pidello, Liliana Vercelli, Federica S. Ricci, Tiziana E. Mongini
Summary: The clinical characteristics of adults with DMD include mechanical ventilation, swallowing and nutritional issues, and bone density alterations. Other issues include respiratory infections, gastrointestinal symptoms, metabolic acidosis, psychiatric symptoms, and chronic pain. Patients have a negative perception of their physical health but a more positive assessment of their mental health.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Review
Biochemistry & Molecular Biology
Ankita Tulangekar, Tamar E. Sztal
Summary: Duchenne muscular dystrophy (DMD) is a severe neuromuscular disorder caused by mutations in the dystrophin gene. Lack of functional dystrophin protein leads to fragile muscle membranes and increased susceptibility to damage during contraction. Inflammation plays a crucial role in exacerbating muscle damage and impairing regeneration in DMD patients, with neutrophils releasing inflammatory compounds that prolong the inflammatory response.
Article
Biochemistry & Molecular Biology
Chantal A. Coles, Ian Woodcock, Daniel G. Pellicci, Peter J. Houweling
Summary: The lack of dystrophin in Duchenne muscular dystrophy results in muscle fragility, inflammation, and subsequent fibrosis. Understanding the involvement of the immune system, especially T cells, in the disease progression is crucial for developing better treatments. This review focuses on the importance of T cells in DMD and suggests targeting T cells as a potential therapeutic approach.
Article
Pharmacology & Pharmacy
Zeren Sun, Dengqiu Xu, Lei Zhao, Xihua Li, Sijia Li, Xiaofei Huang, Chunjie Li, Lixin Sun, Bing Liu, Zhenzhou Jiang, Luyong Zhang
Summary: The study found that fenofibrate can promote the differentiation of myofibers by down-regulating the expression of myostatin protein in myoblasts, significantly improving muscle function and reducing muscle damage in mdx mice, along with anti-inflammatory effects.
BRITISH JOURNAL OF PHARMACOLOGY
(2022)
Article
Biochemistry & Molecular Biology
Keryn G. Woodman, Chantal A. Coles, Shireen R. Lamande, Jason D. White
Summary: Resveratrol at a lower dosage showed potential efficacy in reducing muscle damage and inflammatory cell markers associated with Duchenne muscular dystrophy, suggesting it as a candidate drug for treating DMD.