Article
Biology
Stefania Murzilli, Matteo Serano, Laura Pietrangelo, Feliciano Protasi, Cecilia Paolini
Summary: This study investigates the role of CASQ1 in the postnatal development of muscle fibers. The absence of CASQ1 hinders the full maturation of CRUs and delays the progression of T-tubule biogenesis and organization. These findings highlight the importance of CASQ1 in the development and function of skeletal muscle fibers.
Article
Neurosciences
Barbara Roman, Yusuf Mastoor, Yingfan Zhang, Dennis Gross, Danielle Springer, Chengyu Liu, Brian Glancy, Elizabeth Murphy
Summary: Loss of MICU3 in skeletal muscle leads to impaired exercise capacity and decreased time to fatigue when muscles are directly stimulated. Additionally, MICU3 ablation alters muscle fiber type composition and reduces Ca2+ uptake. These findings demonstrate the important role of MICU3 in skeletal muscle physiology.
JOURNAL OF PHYSIOLOGY-LONDON
(2023)
Review
Biochemistry & Molecular Biology
Antonio Michelucci, Chen Liang, Feliciano Protasi, Robert T. Dirksen
Summary: This review highlights the importance of Ca2+ signaling and ATP generation in skeletal muscle contraction and outlines the role of impaired Ca2+ handling, dysfunctional ROS/RNS production, and structural/functional alterations in CRUs and mitochondria in various muscle disorders. Understanding these processes is crucial for identifying potential therapeutic targets to prevent or reverse muscle dysfunction in aging and disease.
Review
Biochemistry & Molecular Biology
Giorgia Pallafacchina, Sofia Zanin, Rosario Rizzuto
Summary: Advances in scientific research have led to increasing interest in the regulation and physiological role of mitochondrial Ca2+ signaling, with the discovery and characterization of genes and pathways involved in recent years. The identification of the mitochondrial channel components and their function over the past decade has provided a clearer understanding of the role of mitochondrial Ca2+ signals in cellular processes. Recent studies have highlighted the importance of the MCU complex in controlling muscle nutrition and metabolism.
Review
Biochemistry & Molecular Biology
Joao Victor Cabral-Costa, Alicia J. Kowaltowski
Summary: Astrocytes are diverse macroglial cells with various functions and morphologies, containing different types of mitochondria that play important roles in local metabolism and Ca2+ signaling. Ca2+ is crucial for central nervous system processes, and the mitochondrial Ca2+ handling system, including the MCUc, NCLX, and mtPTP, is essential for brain homeostasis and various physiological and pathological processes. This review discusses the molecular composition of the mitochondrial Ca2+ handling system and its impact on astrocytic homeostasis.
ESSAYS IN BIOCHEMISTRY
(2023)
Article
Physiology
Colline Sanchez, Christine Berthier, Yves Tourneur, Laloe Monteiro, Bruno Allard, Laszlo Csernoch, Vincent Jacquemond
Summary: The study utilized a Ca2+-sensitive probe targeted to the junctional SR membrane of intact muscle fibers to investigate the functional properties of RYR-mediated SR Ca2+ release. Results showed spatial gradients of Ca2+ release and remarkable synchronicity of voltage-dependent Ca2+ release activation among individual triads, highlighting the potential of the approach to visualize activation or closure of single groups of RYR channels.
JOURNAL OF GENERAL PHYSIOLOGY
(2021)
Article
Multidisciplinary Sciences
Carlo Manno, Eshwar Tammineni, Lourdes Figueroa, Isabelle Marty, Eduardo Rios
Summary: Triadin plays a key role in regulating calcium signaling and muscle function. Its knockout leads to alterations in calcium balance and changes in the expression of important proteins, such as calsequestrin. However, the muscle cells are able to compensate for these changes and maintain normal muscle function.
Article
Food Science & Technology
Jinjie Li, Yi Li, Xiangying Suo, Jiangtao Li, Da Huang, Guangning Kou
Summary: Bayberry extract has been found to enhance grip strength and metabolic rate in mice, as well as increase the size and percentage of fast-twitch muscle fibers while decreasing slow-twitch fibers. This effect is likely due to its activation of the Akt-FoxO1 pathway. These findings suggest that bayberry extract could be a potential therapy for improving glucose homeostasis in skeletal muscle.
Article
Biochemistry & Molecular Biology
Oh Sung Kwon, Stephen T. Decker, Jia Zhao, John R. Hoidal, Thomas Heuckstadt, Karl A. Sanders, Russell S. Richardson, Gwenael Layec
Summary: This study investigated the role of Advanced Glycation End Products (AGEs) and their receptor (RAGE) in muscle dysfunction with Chronic Obstructive Pulmonary Disease (COPD). The results showed that cigarette smoke exposure caused oxidative stress, downregulated contractile proteins and mitochondrial respiratory complexes, and altered mitochondrial function. Knockout of RAGE protected against oxidative damage and regulated mitochondrial content and function, suggesting it as a potential therapeutic target.
FREE RADICAL BIOLOGY AND MEDICINE
(2023)
Review
Cell Biology
Han Dong, Shih-Yin Tsai
Summary: Mitochondria are crucial for energy production and play a vital role in various biological processes in eukaryotic cells. Skeletal muscle heavily relies on mitochondria for energy supplementation and also relies on them for maintaining calcium and reactive oxygen species levels. This review summarizes recent studies on mitochondria function and quality control in skeletal muscle, focusing on in vivo studies of rodents and human subjects. The interplay between mitochondrial functions and muscle fiber type-specific phenotypes, as well as the impact of aging and exercise on skeletal muscle and mitochondria properties, is also discussed.
Article
Biochemistry & Molecular Biology
Zhengye Liu, Thomas Chaillou, Estela Santos Alves, Theresa Mader, Baptiste Jude, Duarte M. S. Ferreira, Heidi Hynynen, Arthur J. Cheng, William O. Jonsson, Gianluigi Pironti, Daniel C. Andersson, Ellinor Kenne, Jorge L. Ruas, Pasi Tavi, Johanna T. Lanner
Summary: The study demonstrates that NDUFA4L2 regulates skeletal muscle mass and force by decreasing mitochondrial activity and production of reactive oxygen species, leading to muscle atrophy. Overexpression of NDUFA4L2 reduces mitochondrial respiration and antioxidant capacity, lowering levels of important intramuscular metabolites, ultimately affecting muscle function.
Article
Medicine, Research & Experimental
Stephen T. Decker, Alexs A. Matias, Sean T. Bannon, Jack P. Madden, Nadia Alexandrou-Majaj, Gwenael Layec
Summary: Epidemiological and clinical evidence shows that exposure to cigarette smoke affects glucose and fatty acid metabolism, increasing the risk of metabolic disorders. This study aimed to examine the effects of cigarette smoke on mitochondrial respiratory capacity and sensitivity in muscle fibers. The results revealed that cigarette smoke reduced mitochondrial respiratory capacity and sensitivity to pyruvate in the gastrocnemius muscle. Only the sensitivity to pyruvate-stimulated respiration showed a trend towards decrease in the soleus muscle. The findings highlight that cigarette smoke impairs mitochondrial respiration and plays a key role in smoke-induced muscle dysfunction and metabolic disturbances.
Article
Multidisciplinary Sciences
Jun-Ha Hwang, Kyung Min Kim, Ho Taek Oh, Gi Don Yoo, Mi Gyeong Jeong, Hyun Lee, Joori Park, Kwon Jeong, Yoon Ki Kim, Young-Gyu Ko, Eun Sook Hwang, Jeong-Ho Hong
Summary: This study reveals that TAZ is a novel stimulator for mitochondrial biogenesis and exercise-induced muscle adaptation. TAZ stimulates the translation of mitochondrial transcription factor A via the Rheb/Rhebl1-mTOR pathway. The knockout of TAZ in mice results in decreased mitochondrial biogenesis, respiratory metabolism, and exercise ability.
NATURE COMMUNICATIONS
(2022)
Article
Chemistry, Multidisciplinary
Kim Sarah Koeck, Sahar Salehi, Martin Humenik, Thomas Scheibel
Summary: This study focuses on achieving good mechanical stability of collagen fibers through self-assembly during wet-spinning without the need for additional crosslinkers. The produced collagen fibers show fibril alignment and a unique D-band pattern, and are processed into hierarchical assemblies using textile-engineering techniques. These assemblies serve as excellent substrates for muscle microtissue formation, particularly at the muscle-tendon-junction (MTJ) where myoblasts and fibroblasts are co-cultured to develop an MTJ-model.
ADVANCED FUNCTIONAL MATERIALS
(2022)
Article
Nutrition & Dietetics
Jerome Salles, Marine Gueugneau, Veronique Patrac, Carmen Malnero-Fernandez, Christelle Guillet, Olivier Le Bacquer, Christophe Giraudet, Phelipe Sanchez, Marie-Laure Collin, Julien Hermet, Corinne Pouyet, Yves Boirie, Heidi Jacobs, Stephane Walrand
Summary: Aging is associated with muscle loss and decreased muscle function, which increases the risk of mobility limitations and frailty. Dietary interventions with pea proteins and inulin have shown promise in attenuating age-related muscle loss. This study found that supplementing old rats with pea proteins and inulin resulted in preserved muscle mass and improved muscle function.
Article
Biochemistry & Molecular Biology
Martina Scano, Alberto Benetollo, Leonardo Nogara, Michela Bondi, Francesco Dalla Barba, Michela Soardi, Sandra Furlan, Eylem Emek Akyurek, Paola Caccin, Marcello Carotti, Roberta Sacchetto, Bert Blaauw, Dorianna Sandona
Summary: Limb-girdle muscular dystrophy R3 (LGMDR3) is caused by mutations in the SGCA gene, resulting in defective α-sarcoglycan (SG) and muscle weakness. The use of small molecules, such as CFTR correctors, has shown promise in recovering mutated α-SG and restoring muscle function in a novel LGMDR3 murine model, potentially offering a new paradigm for treatment.
HUMAN MOLECULAR GENETICS
(2022)
Article
Food Science & Technology
Giuseppe Sirago, Luana Toniolo, Emanuela Crea, Emiliana Giacomello
Summary: Sarcopenia, a condition that causes muscle loss and functional deficits, starts around the age of 40. A recent study showed that long-term supplementation with Resveratrol can counteract sarcopenia in mice. In a short-term treatment of middle-aged mice with Resveratrol, a decrease in muscle inflammation was observed.
INTERNATIONAL JOURNAL OF FOOD SCIENCES AND NUTRITION
(2022)
Article
Geriatrics & Gerontology
Alessia Geremia, Roberta Sartori, Martina Baraldo, Leonardo Nogara, Valeria Balmaceda, Georgia Ana Dumitras, Stefano Ciciliot, Marco Scalabrin, Hendrik Nolte, Bert Blaauw
Summary: During cancer cachexia, impaired mTORC1 signaling leads to reduced protein synthesis rates in skeletal muscle. Conversely, activation of Akt-mTORC1 signaling can completely reverse muscle loss and force reduction, and prevent the increase in protein degradation observed in muscles from tumor-bearing animals. These findings suggest that skeletal muscle maintains its anabolic capacities during cancer cachexia, potentially explaining the beneficial effects of exercise in cancer patients.
JOURNAL OF CACHEXIA SARCOPENIA AND MUSCLE
(2022)
Article
Neurosciences
Martina Baraldo, Sabrina Zorzato, Achille Homere Tchampda Dondjang, Alessia Geremia, Leonardo Nogara, Ana Georgia Dumitras, Marta Canato, Lorenzo Marcucci, Hendrik Nolte, Bert Blaauw
Summary: This study reveals the functional role of mTOR in muscle contractility. Double knockout of mTOR and raptor in skeletal muscle leads to severe muscle weakness and slower muscle relaxation. The altered mTOR signaling results in increased fiber denervation, changes in gene expression related to calcium, and a shift towards slow-twitch fibers.
JOURNAL OF PHYSIOLOGY-LONDON
(2022)
Editorial Material
Biochemistry & Molecular Biology
Tomas Knedlik, Marta Giacomello
Editorial Material
Physiology
Emiliana Giacomello, Luana Toniolo
FRONTIERS IN PHYSIOLOGY
(2022)
Article
Cell Biology
Francesco Chemello, Michela Pozzobon, Lorenza Iolanda Tsansizi, Tatiana Varanita, Ruben Quintana-Cabrera, Daniele Bonesso, Martina Piccoli, Gerolamo Lanfranchi, Marta Giacomello, Luca Scorrano, Camilla Bean
Summary: Approved gene therapies for spinal muscular atrophy (SMA) improve SMA natural history but are not curative. Loss of survival motor neuron 1 (SMN1) has detrimental effects in muscle beyond motor neurons, leading to accumulation of dysfunctional mitochondria. Amniotic fluid stem cells transplantation can restore mitochondrial morphology and expression of mitochondrial genes in SMA mouse model, suggesting that targeting muscle mitochondrial dysfunction may complement current gene therapy for SMA.
CELL DEATH & DISEASE
(2023)
Article
Cell Biology
Caterina Vianello, Federica Dal Bello, Sang Hun Shin, Sara Schiavon, Camilla Bean, Ana Paula Magalhaes Rebelo, Tomas Knedlik, Emad Norouzi Esfahani, Veronica Costiniti, Rodrigo S. Lacruz, Giuseppina Covello, Fabio Munari, Tommaso Scolaro, Antonella Viola, Elena Rampazzo, Luca Persano, Sara Zumerle, Luca Scorrano, Alessio Gianelle, Marta Giacomello
Summary: Recent studies have found a connection between changes in mitochondria physiology and cellular pathophysiological mechanisms, and the function of these organelles needs to be validated. However, a method for high-throughput quantification of mitochondrial membrane potential has not been established yet. In this study, we present a methodology for unbiased quantification of mitochondrial membrane potential in vitro, suitable for various cell models. Our method successfully analyzed mitochondrial membrane potential in different cell types and allowed discrimination between cell subpopulations using automated image analysis and machine learning.
Article
Biochemistry & Molecular Biology
Stefano Cagnin, Tomas Knedlik, Caterina Vianello, Ana Paula Magalhaes Rebelo, Agnese De Mario, Marta Giacomello
Summary: Neuroblastoma is a common childhood tumor that accounts for 15% of pediatric cancer deaths, but the mechanisms and genes involved in its progression are unknown. In this study, we analyzed the gene expression differences between neuroblastoma stage 1 and stage 4 + 4S to identify biological processes associated with its progression. The results suggest that alterations in mitochondria function and mitochondria-ER contact sites may be involved in the progression of neuroblastoma.
Article
Biology
Giuseppe Sirago, Julian Candia, Martino V. Franchi, Fabio Sarto, Elena Monti, Luana Toniolo, Carlo Reggiani, Emiliana Giacomello, Sandra Zampieri, Lisa M. Hartnell, Giuseppe De Vito, Marco Sandri, Luigi Ferrucci, Marco V. Narici
Summary: Chronic inactivity of skeletal muscles leads to neuromuscular instability and muscle atrophy, and blocking the appearance of specific molecules can reduce the severity of muscle loss. Studies on humans and mice have shown that muscle inactivity causes changes in biomarkers and reveals potential mechanisms associated with neuromuscular disruption.
Review
Nutrition & Dietetics
Luana Toniolo, Monica Concato, Emiliana Giacomello
Summary: Resveratrol, a natural polyphenol, has been utilized in Chinese traditional medicine and is thought to contribute to the French Paradox. Recent studies suggest that resveratrol could mimic the effects of calorie restriction by modulating skeletal muscle metabolism. Although animal studies show potential benefits of resveratrol on skeletal muscle structure and function, clinical trials have not provided clear information. This may be due to the heterogeneity in response to resveratrol in humans and the complexity of skeletal muscle physiology.
Review
Cell Biology
Luana Toniolo, Giuseppe Sirago, Emiliana Giacomello
Summary: Ageing is a complex process involving multiple cellular mechanisms and can lead to the development of various pathologies. The investigation of ageing processes and the search for countermeasures are challenging, requiring the use of experimental models ranging from unicellular organisms to more complex systems. However, these models have their limitations and potential obstacles that need to be considered in ageing research.
HISTOLOGY AND HISTOPATHOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Luana Toniolo, Giuseppe Sirago, Nicola Fiotti, Emiliana Giacomello
Summary: A growing number of disorders are caused by mutations in the vesicular transport machinery, specifically in the Golgi Complex (GC). The GC is crucial for the organization and function of the early secretory pathway, and alterations in its form and function contribute to several disorders, including muscular dystrophies.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Multidisciplinary Sciences
Marta Murgia, Stefano Ciciliot, Nagarjuna Nagaraj, Carlo Reggiani, Stefano Schiaffino, Martino V. Franchi, Rado Pisot, Bostjan Simunic, Luana Toniolo, Bert Blaauw, Marco Sandri, Gianni Biolo, Martin Flueck, Marco V. Narici, Matthias Mann
Summary: Astronauts experience muscle loss, decreased strength, and insulin resistance in space, as well as during prolonged bed rest. By using proteomics, the study reveals molecular remodeling and changes in muscle proteins during bed rest and spaceflight, shedding light on the effects of microgravity and inactivity. Muscle focal adhesions and antioxidant response pathways are downregulated during unloading and restored upon reloading. Additionally, markers of neuromuscular damage are upregulated during unloading.
