Article
Oncology
Christine M. Heske, Yueh-Yun Chi, Rajkumar Venkatramani, Minjie Li, Michael A. Arnold, Roshni Dasgupta, Susan M. Hiniker, Douglas S. Hawkins, Leo Mascarenhas
Summary: In patients with localized, FOXO1 fusion-positive RMS, clinical factors such as age at diagnosis and tumor size can impact survival outcomes, with older age and larger tumors being adverse prognostic factors for EFS. Patients with both older age and large tumor size experience significantly inferior outcomes.
Article
Sport Sciences
Michaela Wehrstein, Axel Schoeffel, Nadine Weiberg, Thomas Gwechenberger, Theresa Betz, Mareike Rittweg, Mario Parstorfer, Maximilian Pilz, Birgit Friedmann-Bette
Summary: The study aimed to assess if one bout of concentric/eccentric exercise with damaging eccentric overload provides a sufficient stimulus to induce satellite cell (SC) activation, proliferation, and differentiation. The results showed that eccentric overload during leg extension exercise induced significant SC activation and increases in SC content, but there were no signs of increased SC differentiation or formation of new myofibers.
MEDICINE & SCIENCE IN SPORTS & EXERCISE
(2022)
Article
Genetics & Heredity
Line J. Manceau, Julien E. Richard Albert, Pier-Luigi J. Lollini, Maxim V. C. E. Greenberg, Pascale J. Gilardi-Hebenstreit, Vanessa E. Ribes
Summary: The chimeric proteins PAX3-FOXO1 and PAX7-FOXO1, derived from chromosomal translocations, exhibit distinct genomic occupancy and transcriptional activity, leading to different pathological manifestations in alveolar rhabdomyosarcoma.
Correction
Biotechnology & Applied Microbiology
Johanna Pruller, Isabella Hofer, Massimo Ganassi, Philipp Heher, Michelle T. Ma, Peter S. Zammit
Summary: The paper has been amended and the revised version can be accessed through a link at the top of the paper.
CANCER GENE THERAPY
(2021)
Article
Biochemistry & Molecular Biology
Mengya Wang, Weihao Song, Chaofan Jin, Kejia Huang, Qianwen Yu, Jie Qi, Quanqi Zhang, Yan He
Summary: Pax3 and Pax7 are crucial for muscle development, with distinct populations of muscle satellite cells in black rockfish marked by SsPax3 and SsPax7. SsPax3a and two SsPax7 genes show increased expression during muscle repair, while SsPax3b remains unchanged, suggesting a more complex muscle stem cell population in teleosts compared to mammals.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Susu Zhang, Jing Wang, Qi Liu, W. Hayes McDonald, Monica L. Bomber, Hillary M. Layden, Jacob Ellis, Scott C. Borinstein, Scott W. Hiebert, Kristy R. Stengel
Summary: Transcriptional control is a dynamic process that is challenging to study using traditional genetic methods. In this study, we used a chemical-genetic approach to quickly degrade the transcriptional activator PAX3-FOXO1 and investigated its mechanism of action. Our findings revealed that PAX3-FOXO1 is involved in regulating gene expression and maintaining chromatin accessibility and enhancer architecture.
Article
Multidisciplinary Sciences
Yuan Wen, Davis A. Englund, Bailey D. Peck, Kevin A. Murach, John J. McCarthy, Charlotte A. Peterson
Summary: The study revealed that satellite cells in muscle are activated during acute exercise, but show limited lineage progression during muscle adaptation. Nuclei without satellite cells demonstrate divergent transcriptional dynamics compared to muscles with sufficient satellite cells.
Article
Pediatrics
Mark C. Xu, M. Owais Ghani, Annie Apple, Heidi Chen, Martin Whiteside, Scott C. Borinstein, Hernan Correa, Harold N. Lovvorn
Summary: This study found that FXR1 expression was universally strong in RMS specimens at diagnosis regardless of disease or patient characteristics, particularly in undifferentiated cells. Reduction in FXR1 expression after chemotherapy was associated with improved survival for embryonal and translocation-negative RMS patients.
JOURNAL OF PEDIATRIC SURGERY
(2021)
Article
Biochemistry & Molecular Biology
Qiande Hu, Liang Zhu, Yuan Li, Jianjun Zhou, Jun Xu
Summary: The study revealed that PAX3-FOXO1 inhibits ACTA1 expression at transcription and protein levels through the RhoA-MKL1-SRF signaling pathway, which may contribute to the tumorigenesis and development of ARMS. Overexpression of ACTA1 in RH30 cells was shown to inhibit cell proliferation and migration in vitro and impair tumor growth in vivo compared to the control groups.
CELL AND BIOSCIENCE
(2021)
Article
Oncology
Daniela Di Carlo, Cyrus Chargari, Jean-Yves Scoazec, Sophie Cotteret, Arthur Felix, Salma Moalla, Stephane Temam, Veronique Minard-Colin
Summary: Alveolar rhabdomyosarcoma (ARMS) is commonly associated with PAX3/PAX7-FOXO1 fusion gene, but some tumors without this fusion may have new molecular alterations. A rare case of PAX3-NCOA1 ARMS in a two-year-old girl with tongue and nodal extension was treated with a combination of therapies to preserve oral function and minimize long-term effects. International collaboration is necessary to evaluate the prognostic value of this variant fusion in ARMS.
PEDIATRIC BLOOD & CANCER
(2021)
Article
Cell Biology
Kwantae Kim, Jameson Orvis, Alberto Stolfi
Summary: Pax3/7 factors play crucial roles in the development of the nervous system in vertebrates and are also involved in similar functions in the model tunicate Ciona. The study reveals that Pax3/7 is expressed in the neural plate borders and central nervous system after neural tube closure, playing a conserved role in neural tube closure and neuron specification. These results suggest that the key functions of Pax3/7 in neural tube development are evolutionarily ancient, dating back to the common ancestor of vertebrates and tunicates.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Article
Health Care Sciences & Services
Jiaqi Bi, Hong Jing, ChenLiang Zhou, Peng Gao, Fujun Han, Gang Li, Shiwei Zhang
Summary: This study found that different magnetic flux densities of low-frequency electromagnetic field (LF-EMF) have varying positive effects on different stages of skeletal myogenesis, including satellite cell activation and proliferation, muscle cell differentiation, and myocyte fusion.
TECHNOLOGY AND HEALTH CARE
(2022)
Article
Pathology
Xinyi Zhang, Shidan Wang, Erin R. Rudzinski, Saloni Agarwal, Ruichen Rong, Donald A. Barkauskas, Ovidiu Daescu, Lauren Furman Cline, Rajkumar Venkatramani, Yang Xie, Guanghua Xiao, Patrick Leavey
Summary: This study developed a computational algorithm for subtype classification and prognosis prediction of rhabdomyosarcoma (RMS) using pathology images. The algorithm achieved good performance in classification and prediction on the test dataset, providing assistance in pathology evaluation and risk stratification.
AMERICAN JOURNAL OF PATHOLOGY
(2022)
Article
Biology
Carla Regina, Ebrahem Hamed, Geoffroy Andrieux, Sina Angenendt, Michaela Schneider, Manching Ku, Marie Follo, Marco Wachtel, Eugene Ke, Ken Kikuchi, Anton G. Henssen, Beat W. Schafer, Melanie Boerries, Amy J. Wagers, Charles Keller, Simone Hettmer
Summary: The study shows that heterogeneous expression of PAX3:FOXO1 at the single cell level in rhabdomyosarcoma cells may provide a critical advantage during tumor progression.
LIFE SCIENCE ALLIANCE
(2021)
Article
Biochemistry & Molecular Biology
Alvaro Centron-Broco, Francesca Rossi, Chiara Grelloni, Raffaele Garraffo, Dario Dattilo, Andrea Giuliani, Gaia Di Timoteo, Alessio Colantoni, Irene Bozzoni, Manuel Beltran Nebot
Summary: In this study, a circular RNA called circAFF1 was found to be overexpressed in alveolar rhabdomyosarcoma. Depletion of circAFF1 was shown to affect cell homeostasis and promote cell migration through the downregulation of genes involved in cell adhesion pathways. These findings highlight the potential of circAFF1 as a future therapeutic target for alveolar rhabdomyosarcoma.
Article
Genetics & Heredity
Mariko Okubo, Satoru Noguchi, Shinichiro Hayashi, Harumasa Nakamura, Hirofumi Komaki, Masafumi Matsuo, Ichizo Nishino
Article
Biotechnology & Applied Microbiology
Aurore Besse, Stephanie Astord, Thibaut Marais, Marianne Roda, Benoit Giroux, Francois-Xavier Lejeune, Frederic Relaix, Piera Smeriglio, Martine Barkats, Maria Grazia Biferi
Article
Cell Biology
Jennyfer Levoux, Alexandre Prola, Peggy Lafuste, Marianne Gervais, Nathalie Chevallier, Zeynab Koumaiha, Kaouthar Kefi, Laura Braud, Alain Schmitt, Azzedine Yacia, Aurelie Schirmann, Barbara Hersant, Mounia Sid-Ahmed, Sabrina Ben Larbi, Katerina Komrskova, Jakub Rohlena, Frederic Relaix, Jiri Neuzil, Anne-Marie Rodriguez
Summary: Platelets enhance the therapeutic potential of MSCs by transferring mitochondria and promoting pro-angiogenic activity. Activation of the de novo fatty acid synthesis pathway is required for increased secretion of pro-angiogenic factors by platelet-preconditioned MSCs. Testing platelet mitochondria quality before clinical use is crucial for optimizing their effects.
Article
Rheumatology
Cyrielle Hou, Chloe Durrleman, Baptiste Periou, Christine Barnerias, Christine Bodemer, Isabelle Desguerre, Pierre Quartier, Isabelle Melki, Gillian I. Rice, Mathieu P. Rodero, Jean-Luc Charuel, Frederic Relaix, Brigitte Bader-Meunier, Francois Jerome Authier, Cyril Gitiaux
Summary: The expression of ISG15 is closely associated with juvenile DM, with patients exhibiting different ISG15 muscle signatures according to their MSA class. Patients with juvenile DM who are positive for MDA-5 have higher levels of ISG15 expression in both gene and protein form compared to other subgroups. Additionally, the data shows that negative regulation of type I IFN correlates with milder muscle involvement.
ARTHRITIS & RHEUMATOLOGY
(2021)
Article
Multidisciplinary Sciences
Alexandre Prola, Jordan Blondelle, Aymeline Vandestienne, Jerome Piquereau, Raphael G. P. Denis, Stephane Guyot, Hadrien Chauvin, Arnaud Mourier, Marie Maurer, Celine Henry, Nahed Khadhraoui, Cindy Gallerne, Thibaut Molinie, Guillaume Courtin, Laurent Guillaud, Melanie Gressette, Audrey Solgadi, Florent Dumont, Julien Castel, Julien Ternacle, Jean Demarquoy, Alexandra Malgoyre, Nathalie Koulmann, Genevieve Derumeaux, Marie-France Giraud, Frederic Joubert, Vladimir Veksler, Serge Luquet, Frederic Relaix, Laurent Tiret, Fanny Pilot-Storck
Summary: Mice deficient for a muscle-specific enzyme of very-long-chain fatty acid synthesis displayed increased basal energy expenditure and protection against high-fat diet-induced obesity. Muscle-specific modulation of the very-long-chain fatty acid pathway was associated with a reduced content of inner mitochondrial membrane phospholipid cardiolipin and a blunted coupling efficiency between the respiratory chain and ATP synthase, which was restored by cardiolipin enrichment. Selective increase of lipid oxidative capacities in skeletal muscle, through the cardiolipin-dependent lowering of mitochondrial ATP production, provides an effective option against obesity at the whole-body level.
Correction
Cell Biology
Jennyfer Levoux, Alexandre Prola, Peggy Lafuste, Marianne Gervais, Nathalie Chevallier, Zeynab Koumaiha, Kaouthar Kefi, Laura Braud, Alain Schmitt, Azzedine Yacia, Barbara Hersant, Mounia Sid-Ahmed, Sabrina Ben Larbi, Katerina Komrskova, Jakub Rohlena, Frederic Relaix, Jiri Neuzil, Anne-Marie Rodriguez
Article
Cell & Tissue Engineering
Leo Machado, Perla Geara, Jordi Camps, Matthieu Dos Santos, Fatima Teixeira-Clerc, Jens Van Herck, Hugo Varet, Rachel Legendre, Jean-Michel Pawlotsky, Maurilio Sampaolesi, Thierry Voet, Pascal Maire, Frederic Relaix, Philippos Mourikis
Summary: Tissue damage alters cell-microenvironment interactions, influencing cells' responses. Muscle stem cell activation after injury serves as a paradigm of cell activation, while a broadly conserved stress response is shared across different cell types.
Review
Multidisciplinary Sciences
F. Relaix, M. Bencze, M. J. Borok, A. Der Vartanian, F. Gattazzo, D. Mademtzoglou, S. Perez-Diaz, A. Prola, P. C. Reyes-Fernandez, A. Rotini, V Taglietti
Summary: Skeletal muscle possesses remarkable regenerative capabilities, mainly attributed to its resident muscle stem cells. Recent technological advancements have provided mechanistic insights into MuSC biology, emphasizing their role in regeneration, muscle disorders, and aging. The complex regulation of MuSCs by their niche and the understanding of their metabolic role impact the broader understanding of stem cells and their therapeutic applications in regenerative medicine.
NATURE COMMUNICATIONS
(2021)
Article
Multidisciplinary Sciences
Anais Julien, Anuya Kanagalingam, Ester Martinez-Sarra, Jerome Megret, Marine Luka, Mickael Menager, Frederic Relaix, Celine Colnot
Summary: The study highlights the essential role of tissue resident stem cells in bone regeneration and the significant contribution of mesenchymal progenitors from skeletal muscle to fibrotic response and bone repair. Impaired processes are observed when both bone and skeletal muscle are injured.
NATURE COMMUNICATIONS
(2021)
Article
Cell Biology
Matthew Borok, Nathalie Didier, Francesca Gattazzo, Teoman Ozturk, Aurelien Corneau, Helene Rouard, Frederic Relaix
Summary: The study utilized mass cytometry to track changes in different cell types during muscle regeneration, revealing rapid proteomic changes in various cell types following early activation. After injury induction, there is a drastic reduction in myogenic, vascular, and mesenchymal cell lineages, while immune cells invade the damaged tissue significantly.
Article
Cell Biology
Joana Esteves de Lima, Reem Bou Akar, Myriam Mansour, Didier Rocancourt, Margaret Buckingham, Frederic Relaix
Summary: PAX3, a transcription factor in the paired-homeobox family, plays a crucial role in regulating muscle progenitor cells during embryonic development. Mutations in Pax3 can impair myotome formation, especially when combined with Pax3 null and Pax3 dominant-negative alleles. The adhesion molecule M-Cadherin is identified as a PAX3 target gene linked to myotome formation.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Multidisciplinary Sciences
Joana Esteves de Lima, Reem Bou Akar, Leo Machado, Yuefeng Li, Bernadette Drayton-Libotte, F. Jeffrey Dilworth, Frederic Relaix
Summary: The authors demonstrate that HIRA, a H3.3 histone chaperone, establishes the chromatin landscape required for skeletal muscle cell identity.
NATURE COMMUNICATIONS
(2021)
Review
Biochemistry & Molecular Biology
Joana Esteves de Lima, Frederic Relaix
Summary: This passage discusses the importance of epigenetic regulation of gene expression in maintaining cell differentiation status and cellular identity, focusing on chromatin structure remodeling and changes in nucleosome composition. It emphasizes the role of histone post-translational modifications and histone variants in cell fate and differentiation.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Cell Biology
Leo Machado, Frederic Relaix, Philippos Mourikis
Summary: The rapid advancement of single-cell RNA-sequencing technology has made it possible to generate comprehensive transcriptional maps of nearly all cells in multicellular tissues. Key elements such as scalability, spatial information, and accuracy of individual cell transcriptomes are critical in this process. Traditional cell-isolation protocols and new methods have been discussed in terms of capturing the transcriptional signatures of cells in their native tissue context, revealing new insights into biological processes.
TRENDS IN CELL BIOLOGY
(2021)
Article
Cell Biology
Marie-Luce Vignais, Jennyfer Levoux, Pierre Sicard, Khattar Khattar, Catherine Lozza, Marianne Gervais, Safia Mezhoud, Jean Nakhle, Frederic Relaix, Onnik Agbulut, Jeremy Fauconnier, Anne-Marie Rodriguez
Summary: Preconditioning MSCs with cardiac mitochondria can enhance their therapeutic potential for ischemic heart disease.
