Article
Cell & Tissue Engineering
Lynn Yap, Li Yen Chong, Clarissa Tan, Swarnaseetha Adusumalli, Millie Seow, Jing Guo, Zuhua Cai, Sze Jie Loo, Eric Lim, Ru San Tan, Elina Grishina, Poh Loong Soong, Narayan Lath, Lei Ye, Enrico Petretto, Karl Tryggvason
Summary: Stem cell-derived committed cardiac progenitors (CCPs) showed potential in regenerative cardiology, as observed in a study where pluripotent stem cells were differentiated into CCPs and transplanted into infarcted pig hearts, resulting in significant improvement in heart function and reduction in infarction size.
NPJ REGENERATIVE MEDICINE
(2023)
Article
Biochemistry & Molecular Biology
Katarina Gros, Urska Matkovic, Giulia Parato, Katarina Mis, Elisa Luin, Annalisa Bernareggi, Marina Sciancalepore, Tomaz Mars, Paola Lorenzon, Sergej Pirkmajer
Summary: Neuronal agrin has an age-dependent role in promoting the proliferation of human myoblasts by binding to Lrp4 and activating MuSK.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Cell & Tissue Engineering
Yuki Kihara, Jun Homma, Ryo Takagi, Keiko Ishigaki, Satoru Nagata, Masayuki Yamato
Summary: This study presents a novel and cost-effective method for the primary culture of myoblasts obtained from mouse and rat skeletal muscle, utilizing the high affinity of integrin a7X2b1 to laminin-221.
REGENERATIVE THERAPY
(2022)
Article
Cell Biology
Kaizhi Ding, Yanchun Gong, Chunfang Cheng, Xiaonuo Li, Yuanjie Zhu, Xiaofei Gao, Yuhua Li, Chunhua Yuan, Zhibing Liu, Wei Jiang, Chong Chen, Li-Hua Yao
Summary: This study investigates the role of voltage-gated sodium channels (VGSC) in muscle regeneration. The findings suggest that with the progression of differentiation, VGSC expression and intracellular sodium concentration increase, and VGSC dynamics change, potentially contributing to muscle contraction.
CELLULAR SIGNALLING
(2024)
Article
Engineering, Biomedical
Fernanda Thome Brochado, Belkiss Camara Marmora, Paloma Santos Campos, Tuany Rafaeli Schmidt, Kristianne Porta Santos Fernandes, Sandra Kalil Bussadori, Lucas Goncalves Santos, Vivian Petersen Wagner, Marcelo Lazzaron Lamers, Manoela Domingues Martins
Summary: The present study analyzed the effect of photobiomodulation therapy (PBMT) using defocused high-power laser (DHPL) on myoblast cell migration and compared it with low-power laser therapy. The results showed that PBMT could accelerate the migration of C2C12 myoblast cells, with the dual-wavelength 2 W protocol being the most effective, while having little impact on cell viability.
LASERS IN MEDICAL SCIENCE
(2022)
Article
Pharmacology & Pharmacy
Gerda M. Sanvee, Jamal Bouitbir, Stephan Krahenbuhl
Summary: Simvastatin has different toxic effects on myoblasts and myotubes, with a stronger impact on myoblasts. It impairs myoblast proliferation and myotube formation, potentially representing a new mechanism of statin myotoxicity.
BIOCHEMICAL PHARMACOLOGY
(2021)
Article
Biology
Masashi Fukuoka, Hiromi Fujita, Kosumo Numao, Yasuko Nakamura, Hideo Shimizu, Masayuki Sekiguchi, Hirohiko Hohjoh
Summary: The study reveals that cell-free miR-199-3p in the blood is significantly decreased in aged mice compared to young mice, and supplementing it can enhance muscle regeneration and improve muscle strength. This finding holds potential for hypertrophic effect in aged muscle fibers and as a novel RNA therapeutic for diseases like DMD.
COMMUNICATIONS BIOLOGY
(2021)
Review
Biochemistry & Molecular Biology
Amelia Eva Aranega, Estefania Lozano-Velasco, Lara Rodriguez-Outeirino, Felicitas Ramirez de Acuna, Diego Franco, Francisco Hernandez-Torres
Summary: miRNAs play a critical role in modulating muscle regeneration and stem cell behavior, which is essential for treating muscle disorders. Recent advancements suggest that optimizing muscle stem cell response through miRNAs, in conjunction with gene replacement therapies, can improve muscle regeneration in the context of DMD.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Yanwen Liu, Yilong Yao, Yongsheng Zhang, Chao Yan, Mingsha Yang, Zishuai Wang, Wangzhang Li, Fanqinyu Li, Wei Wang, Yalan Yang, Xinyun Li, Zhonglin Tang
Summary: This study aimed to discover the regulatory function of miR-200c-5p in skeletal muscle regeneration. The results showed that miR-200c-5p increased at the early stage of mouse skeletal muscle regeneration and peaked on the first day. Overexpression of miR-200c-5p promoted migration and inhibited differentiation of C2C12 myoblasts, while inhibition of miR-200c-5p had the opposite effect. It was found that Adamts5 is a target gene of miR-200c-5p and their expression patterns were opposite during skeletal muscle regeneration.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Cell Biology
Iwona Bronisz-Budzynska, Magdalena Kozakowska, Katarzyna Pietraszek-Gremplewicz, Magdalena Madej, Alicja Jozkowicz, Agnieszka Loboda, Jozef Dulak
Summary: Increased oxidative stress can hinder skeletal muscle regeneration and affect muscle satellite cells (mSCs) activity. NRF2 transcription factor plays an important role in muscle cell biology by promoting myoblast proliferation and viability, reducing reactive oxygen species production, and inhibiting myoblast differentiation. NRF2 is also crucial for the viability of mSCs.
Article
Biochemistry & Molecular Biology
Alec Wright, Arielle Hall, Tara Daly, Tatiana Fontelonga, Sarah Potter, Caitlin Schafer, Andrew Lindsley, Christina Hung, Olaf Bodamer, Emanuela Gussoni
Summary: Kabuki syndrome is a rare genetic disorder caused by mutations in histone modifier genes, leading to complex phenotypes. Research on the consequences of loss of KMT2D function in muscles of mice and humans revealed that epigenetic changes associated with the disorder can be reversed in a suitable physiological environment.
Article
Physiology
Misa Horiike, Yoshiko Ogawa, Shigeo Kawada
Summary: Both hyperoxic and hypoxic conditions directly enhance myoblast proliferation, but do not affect differentiation efficiency in C2C12 cells. Macrophages may mediate the acceleration of muscle regeneration under hyperoxic conditions. Excess oxidative stress can abolish the enhancement of myoblast proliferation induced by hyperoxia.
AMERICAN JOURNAL OF PHYSIOLOGY-REGULATORY INTEGRATIVE AND COMPARATIVE PHYSIOLOGY
(2021)
Article
Multidisciplinary Sciences
Veronica Pini, Virginie Mariot, Julie Dumonceaux, John Counsell, Helen C. O'Neill, Sarah Farmer, Francesco Conti, Francesco Muntoni
Summary: By using CRISPR/Cas9, this study demonstrates the restoration of DMD duplications and reveals that high transient expression of Cas9 can bypass the requirement of continuous expression, providing important insights for future therapeutic approaches for in vivo dystrophin restoration.
SCIENTIFIC REPORTS
(2022)
Article
Multidisciplinary Sciences
Michael J. Stec, Qi Su, Christina Adler, Lance Zhang, David R. Golann, Naveen P. Khan, Lampros Panagis, S. Armando Villalta, Min Ni, Yi Wei, Johnathon R. Walls, Andrew J. Murphy, George D. Yancopoulos, Gurinder S. Atwal, Sandra Kleiner, Gabor Halasz, Mark W. Sleeman
Summary: Using spatial transcriptomics and single-cell RNA sequencing datasets, a high-resolution cellular and molecular spatial atlas of the severely dystrophic D2-mdx mouse model was generated. Clustering analysis revealed the nonuniform distribution of unique cell populations associated with multiple regenerative timepoints, faithfully recapitulating the asynchronous regeneration observed in human DMD muscle. Through spatiotemporal gene expression signatures, it was found that propagation of inflammatory and fibrotic signals from locally damaged areas contributes to widespread pathology and identifying targetable pathways for DMD therapy within discrete microenvironments. Overall, this spatial atlas of dystrophic muscle provides a valuable resource for studying DMD disease biology and therapeutic target discovery.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2023)
Review
Cell Biology
Shanshan Yao, Zihao Chen, Yuanyuan Yu, Ning Zhang, Hewen Jiang, Ge Zhang, Zongkang Zhang, Baoting Zhang
Summary: Duchenne muscular dystrophy is a lethal neuromuscular disorder caused by the absence of dystrophin protein, with no cure currently available. The standard of care involves glucocorticoids treatments for symptom relief. Therapeutic strategies focus on restoring dystrophin function and targeting downstream pathological changes like inflammation and fibrosis.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Infectious Diseases
Ingo Riederer, Daniella Areas Mendes-da-Cruz, Guilherme Cordenonsi da Fonseca, Mariela Natacha Gonzalez, Otavio Brustolini, Cassia Rocha, Guilherme Loss, Joseane Biso de Carvalho, Mariane Talon Menezes, Lidiane Menezes Souza Raphael, Alexandra Gerber, Myrna Cristina Bonaldo, Gillian Butler-Browne, Vincent Mouly, Vinicius Cotta-de-Almeida, Wilson Savino, Ana Tereza Ribeiro de Vasconcelos
Summary: This study investigated the mechanisms of Zika virus infection in human skeletal muscle using an in vitro model. The research found that myoblasts are permissive to ZIKV infection, while myotubes control viral replication. Gene expression profiling revealed differences between infected myoblasts and myotubes, with the latter showing pathways related to antiviral and innate immune responses. This study sheds light on potential antiviral mechanisms against ZIKV infection in skeletal muscle.
PLOS NEGLECTED TROPICAL DISEASES
(2022)
Article
Biochemistry & Molecular Biology
Gustavo Silva-Oliveira, Leandra Linhares-Lacerda, Thayana R. F. Mattos, Camila Sanches, Tatiana Coelho-Sampaio, Ingo Riederer, Elvira M. Saraiva
Summary: Neutrophils release neutrophil extracellular traps (NETs) when they interact with different laminin (LM) isoforms. The induction of NETs by laminins is partially inhibited by anti-alpha 6 integrin antibody and requires elastase and peptidylarginine deiminase (PAD)-4. Laminins also enhance the release of NETs triggered by a protozoan parasite and exhibit leishmanicidal activity.
Article
Cell Biology
Ekaterina Kiseleva, Olesya Serbina, Anna Karpukhina, Vincent Mouly, Yegor S. Vassetzky
Summary: Facioscapulohumeral muscular dystrophy (FSHD) is a genetic disease caused by the ectopic expression of the DUX4 gene in skeletal muscle, leading to muscle degeneration and replacement by fat and connective tissue. This study demonstrates that the interaction between FSHD myoblasts and MSCs plays a crucial role in the pathophysiology of FSHD, including stimulation of MSC migration, proliferation, and collagen secretion.
JOURNAL OF CELLULAR PHYSIOLOGY
(2022)
Article
Geriatrics & Gerontology
Mona Bensalah, Laura Muraine, Alexis Boulinguiez, Lorenzo Giordani, Victorine Albert, Victor Ythier, Jamila Dhiab, Alison Oliver, Valentine Hanique, Teresa Gidaro, Sophie Perie, Jean Lacau St-Guily, Aurelien Corneau, Gillian Butler-Browne, Anne Bigot, Vincent Mouly, Elisa Negroni, Capucine Trollet
Summary: This study explored the role of nonmyogenic cells (fibroadipogenic progenitors, FAPs) in human fibrotic muscles and found that FAPs from fibrotic muscles have a higher proliferation rate compared to those from nonfibrotic muscles, which leads to impaired fusion index when cocultured with muscle cells. The study also identified endothelin as a potential target to counteract human muscle fibrosis through a paracrine signaling pathway.
JOURNAL OF CACHEXIA SARCOPENIA AND MUSCLE
(2022)
Article
Cell Biology
Jaione Lasa-Elgarresta, Laura Mosqueira-Martin, Klaudia Gonzalez-Imaz, Pablo Marco-Moreno, Gorka Gerenu, Kamel Mamchaoui, Vincent Mouly, Adolfo Lopez de Munain, Ainara Vallejo-Illarramendi
Summary: LGMDR1 is caused by mutations in the CAPN3 gene, leading to reduced SERCA levels. In this study, researchers found that inhibiting the ubiquitin-proteasome pathway can increase SERCA levels and potentially rescue mutant CAPN3 protein. However, treatment with a proteasome inhibitor did not effectively restore SERCA levels in an animal model.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Review
Immunology
Alexandra Jade Thawley, Luciana Peixoto Veneziani, Francisco Diego Rabelo-da-Ponte, Ingo Riederer, Daniella Areas Mendes-da-Cruz, Victorio Bambini-Junior
Summary: Autism spectrum disorder (ASD) is a neurodevelopmental disorder characterized by stereotyped behaviors, specific interests, and impaired communication skills. Elevated levels of pro-inflammatory cytokine IL-17A (IL-17) have been implicated as part of immune alterations in ASD. Studies using rodent models have shown the role of T-cell activation and IL-17 secretion in the pathogenesis of ASD. A systematic review of 28 studies on IL-17 levels in rodent models of ASD found that IL-17 levels were generally increased in different ASD models.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Clinical Neurology
Fanny Roth, Jamila Dhiab, Alexis Boulinguiez, Hadidja-Rose Mouigni, Saskia Lassche, Elisa Negroni, Laura Muraine, Alix Marhic, Alison Oliver, Jeanne Laine, Andree Rouche, Erin K. O'Ferrall, Baziel van Engelen, Coen Ottenheijm, Hagar Greif, Sergiu Blumen, Jean Lacau St Guily, Sophie Perie, Gillian Butler-Browne, Vincent Mouly, Capucine Trollet
Summary: In this study, the authors conducted a comprehensive analysis of PABPN1 aggregates in human muscle biopsy samples. They found that age and genotype influence the formation of PABPN1 aggregates, and identified new components of these aggregates. They also observed larger aggregates in myonuclei and fewer aggregates in the pharyngeal muscle. In addition, they confirmed the presence of PABPN1 aggregates during muscle fiber regeneration using a xenograft model. These findings contribute to our understanding of the pathophysiology of OPMD.
ACTA NEUROPATHOLOGICA
(2022)
Correction
Cell Biology
Leandro Ladislau, Debora M. Portilho, Tristan Courau, Alhondra Solares-Perez, Elisa Negroni, Jeanne Laine, David Klatzmann, Adriana Bonomo, Yves Allenbach, Olivier Benveniste, Ingo Riederer, Wilson Savino, Vincent Mouly, Gillian Butler-Browne, Claudia F. Benjamim
CELL DEATH & DISEASE
(2022)
Article
Cell Biology
Elisa Negroni, Maria Kondili, Laura Muraine, Mona Bensalah, Gillian Sandra Butler-Browne, Vincent Mouly, Anne Bigot, Capucine Trollet
Summary: Skeletal muscle is a highly plastic tissue that participates in the maintenance, regeneration, and repair of muscles through the interaction and communication of heterogeneous cell populations. Fibro-Adipogenic Progenitors (FAPs) are crucial in muscle homeostasis and regeneration, as they interact with other cells and dynamically remodel the extracellular matrix.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Article
Oncology
Anton De Spiegeleer, Evelien Wynendaele, Amelie Descamps, Nathan Debunne, Bart P. Braeckman, Marjan De Mey, Julie Coudenys, Liesbeth Crombez, Frederick Verbeke, Yorick Janssens, Rekin's Janky, Evy Goossens, Caroline Vlaeminck, Dries Duchi, Vanessa Andries, Emilie Dumas, Mirko Petrovic, Tom van de Wiele, Daniel Knappe, Ralf Hoffmann, Vincent Mouly, Anne Bigot, Lars Vereecke, Filip Van Immerseel, Nele van den Noortgate, Bart De Spiegeleer, Dirk Elewaut
CLINICAL AND TRANSLATIONAL MEDICINE
(2022)
Article
Pharmacology & Pharmacy
Laura Muraine, Mona Bensalah, Gillian Butler -Browne, Anne Bigot, Capucine Trollet, Vincent Mouly, Elisa Negroni
Summary: Fibrosis, the excessive accumulation of extracellular matrix, occurs in many muscle disorders and interferes with muscle regeneration and gene therapies. Slowing down or reversing fibrosis is crucial for maintaining muscle function. Various therapeutic compounds targeting fibrogenic signals have been tested in muscle diseases, particularly in Duchenne Muscular Dystrophy. This review provides an overview of pharmacotherapies tested to reduce fibrosis in skeletal muscle.
CURRENT OPINION IN PHARMACOLOGY
(2023)
Article
Medicine, Research & Experimental
Alexis Boulinguiez, Fany Roth, Hadidja Rose Mouigni, Gillian Butler-Browne, Vincent Mouly, Capucine Trollet
Summary: This study focuses on the basic research of oculopharyngeal muscular dystrophy (OPMD), which is a disease caused by trinucleotide expansions. The presence of aggregates within muscle fiber nuclei is believed to play a crucial role in the pathogenesis of OPMD. Understanding their composition and deleterious effects can lead to new therapeutic approaches.
M S-MEDECINE SCIENCES
(2023)
Article
Multidisciplinary Sciences
Wonyul Jang, Dmytro Puchkov, Paula Samso, YongTian Liang, Michal Nadler-Holly, Stephan J. Sigrist, Ulrich Kintscher, Fan Liu, Kamel Mamchaoui, Vincent Mouly, Volker Haucke
Summary: Cells respond to fluctuating nutrient supply by changing organelle dynamics and metabolism. This study reveals that lipid signaling on the endoplasmic reticulum can control mitochondrial morphology and function, enabling cells to adapt to changing nutrient environments.
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)