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Muscle-skeletal chains

Paola Mantuano, Francesca Sanarica, Elena Conte, Maria Grazia Morgese, Roberta Francesca Capogrosso, Anna Cozzoli, Adriano Fonzino, Angelo Quaranta, Jean-Francois Rolland, Michela De Bellis, Giulia Maria Camerino, Luigia Trabace, Annamaria De Luca
The pharmacological stimulation of AMP-activated protein kinase (AMPK) via metabolic enhancers has been proposed as potential therapeutic strategy for Duchenne muscular dystrophy (DMD). Metformin, a widely-prescribed anti-hyperglycemic drug which activates AMPK via mitochondrial respiratory chain, has been recently tested in DMD patients in synergy with nitric oxide (NO)-precursors, with encouraging results. However, preclinical data supporting the use of metformin in DMD are still poor, and its actions on skeletal muscle appear controversial...
April 20, 2018: Biochemical Pharmacology
Gilles Gouspillou, Richard Godin, Jérome Piquereau, Martin Picard, Mahroo Mofarrahi, Jasmin Mathew, Fennigje M Purves-Smith, Nicolas Sgarioto, Russell T Hepple, Yan Burelle, Sabah Na Hussain
KEY POINTS SUMMARY: Parkin, an E3 ubiquitin ligase encoded by the Park2 gene, has been implicated in the regulation of mitophagy, a quality control process whereby defective mitochondria are degraded. The exact physiological significance of Parkin in regulating mitochondrial function and contractility in skeletal muscle remains largely unexplored. Using Park2-/- mice, we show that Parkin ablation causes a decrease in muscle specific force, a severe decrease in mitochondrial respiration, mitochondrial uncoupling and an increased susceptibility to opening of the permeability transition pore...
April 22, 2018: Journal of Physiology
Cynthia M F Monaco, Meghan C Hughes, Sofhia V Ramos, Nina E Varah, Christian Lamberz, Fasih A Rahman, Chris McGlory, Mark A Tarnopolsky, Matthew P Krause, Robert Laham, Thomas J Hawke, Christopher G R Perry
AIMS/HYPOTHESIS: A comprehensive assessment of skeletal muscle ultrastructure and mitochondrial bioenergetics has not been undertaken in individuals with type 1 diabetes. This study aimed to systematically assess skeletal muscle mitochondrial phenotype in young adults with type 1 diabetes. METHODS: Physically active, young adults (men and women) with type 1 diabetes (HbA1c 63.0 ± 16.0 mmol/mol [7.9% ± 1.5%]) and without type 1 diabetes (control), matched for sex, age, BMI and level of physical activity, were recruited (n = 12/group) to undergo vastus lateralis muscle microbiopsies...
April 18, 2018: Diabetologia
Francine Parker, Matthew Batchelor, Marcin Wolny, Ruth Hughes, Peter J Knight, Michelle Peckham
Over 20 mutations in β-cardiac myosin heavy chain (β-MHC), expressed in cardiac and slow muscle fibres, cause Laing early onset distal myopathy (MPD-1), a skeletal muscle myopathy. Most of these mutations are in the coiled-coil tail, and commonly involve a mutation to a proline, or a single residue deletion, both of which are predicted to strongly affect the secondary structure of the coiled coil. To test this, we characterised the effects of two MPD-1 causing mutations: A1603P and K1617del in vitro and in cells...
April 13, 2018: Journal of Molecular Biology
Andrea C Crupkin, Fernando G Iturburu, Marcos Crupkin, Mirta L Menone
Endosulfan (ES) modifies the ultrastructure of skeletal muscle fibers and causes changes to the swimming behavior of fish. The objectives of the present work were to evaluate, in fishes of Australoheros facetus, 1) the integrity of myofibrils (Mf) by the analysis of SDS-PAGE profiles, and 2) the functionality of Mf through the microscopically monitoring of the contraction and changes in Mg2+ Ca2+ - ATPase and Mg2+ (EGTA) -ATPase activities. As expected, after the addition of the contraction buffer, control fish Mf contracted...
April 12, 2018: Ecotoxicology and Environmental Safety
Veronika Boczonadi, Kathrin Meyer, Humberto Gonczarowska-Jorge, Helen Griffin, Andreas Roos, Marina Bartsakoulia, Boglarka Bansagi, Giulia Ricci, Fanni Palinkas, René P Zahedi, Francesco Bruni, Brian Kaspar, Hanns Lochmüller, Kym M Boycott, Juliane S Müller, Rita Horvath
The nuclear-encoded glycyl-tRNA synthetase gene (GARS) is essential for protein translation in both cytoplasm and mitochondria. In contrast, different genes encode the mitochondrial and cytosolic forms of most other tRNA synthetases. Dominant GARS mutations were described in inherited neuropathies, while recessive mutations cause severe childhood-onset disorders affecting skeletal muscle and heart. The downstream events explaining tissue-specific phenotype-genotype relations remained unclear. We investigated the mitochondrial function of GARS in human cell lines and in the GarsC210R mouse model...
April 10, 2018: Human Molecular Genetics
Jan F C Glatz, Joost J F P Luiken
The widely expressed transmembrane glycoprotein CD36, a scavenger receptor class B protein (SR-B2), serves many functions in lipid metabolism and signaling. Here, we review CD36's role in facilitating cellular long-chain fatty-acid uptake across the plasma membrane, particularly in heart and skeletal muscles. CD36 acts in concert with other membrane proteins, such as peripheral plasma membrane fatty acid-binding protein (FABPpm), and is an intracellular docking site for cytoplasmic FABP (FABPc). The cellular fatty-acid uptake rate is governed primarily by the presence of CD36 at the cell surface, which is regulated by the subcellular vesicular recycling of CD36 from endosomes to the plasma membrane...
April 7, 2018: Journal of Lipid Research
HuiLi Tong, RunYing Jiang, TingTing Liu, Yao Wei, ShuFeng Li, YunQin Yan
The mechanism by which bta-miR-378 regulates bovine skeletal muscle-derived satellite cell (bMDSC) myogenesis remains unknown. In this study, stem-loop RT-PCR was used to assess bta-miR-378 expression during the proliferation and differentiation of bMDSCs. The results showed that bta-miR-378 expression did not obviously change during bMDSC proliferation but increased significantly when bMDSCs began to differentiate. Then, a bta-miR-378 mimic (bta-miR-378-M) and bta-miR-378 inhibitor (bta-miR-378-I) were transfected into bMDSCs to explore the effect of bta-miR-378 on bMDSC differentiation...
April 2, 2018: Gene
Vito Errico, Giuseppe Arrabito, Ersilia Fornetti, Claudia Fuoco, Stefano Testa, Giovanni Saggio, Stefano Rufini, Stefano Maria Cannata, Alessandro Desideri, Christian Falconi, Cesare Gargioli
Mesoangioblasts are outstanding candidates for stem cell therapy and are already being explored in clinical trials. However, a crucial challenge in regenerative medicine is the limited availability of undifferentiated myogenic progenitor cells, since growth is typically accompanied by differentiation. Here reversible myogenic-differentiation-switching during proliferation is achieved by functionalizing the glass substrate with high-density ZnO nanowires. Specifically, mesoangioblasts grown on ZnO nanowires present a spherical viable undifferentiated cell state without lamellopodia formation during all the observation time (8 days)...
April 5, 2018: ACS Applied Materials & Interfaces
Donald K Layman, Bo Lönnerdal, John D Fernstrom
α-Lactalbumin is a whey protein that constitutes approximately 22% of the proteins in human milk and approximately 3.5% of those in bovine milk. Within the mammary gland, α-lactalbumin plays a central role in milk production as part of the lactose synthase complex required for lactose formation, which drives milk volume. It is an important source of bioactive peptides and essential amino acids, including tryptophan, lysine, branched-chain amino acids, and sulfur-containing amino acids, all of which are crucial for infant nutrition...
March 29, 2018: Nutrition Reviews
Sergio Fabris, David A MacLean
Skeletal muscle (SM) health and integrity is dependent on the dynamic balance between protein synthesis and degradation, and central to this process is the availability of amino acids (AA) in the amino pool. While Doxorubicin (DOX) remains one of the most widely used chemotherapeutic agents for the treatment of solid and hematological malignancies, little is known of the effect of the drug on SM, particularly its effect on the availability of amino acids in the tissue. The purpose of this study was to examine the effect of DOX administration on vascular, interstitial and intracellular concentrations of AA in SM of the rat up to 8 days after the administration of a 1...
2018: PloS One
Ana S H Costa, Paulo Costa, Susana P Alves, Cristina M Alfaia, José A M Prates, Veronica Vleck, Isabelle Cassar-Malek, Jean-François Hocquette, Rui J B Bessa
Despite the recent advances in transcriptomics, gene expression studies addressing cattle´s skeletal muscle adaptations in response to compensatory growth are warranted, particularly regarding lipid metabolism due to its impact in meat sensory and nutritional traits. In the present study, in comparison to ad libitum feeding, a period of feed restriction was used in order to understand the changes in bull´s lipid metabolism and gene expression of the adipogenic and lipogenic pathways after re-alimentation...
2018: PloS One
Sang-Guk Lee, Ye Seal Yim, Yong-Ho Lee, Byung-Wan Lee, Hyon-Suk Kim, Kyung-Sup Kim, Yong-Wha Lee, Jeong-Ho Kim
AIMS: We evaluated specific alterations in amino acids (AAs) profile in patients with type 2 diabetes mellitus (T2DM) and impaired fasting glucose (IFG) compared with healthy controls. In addition, we tried to find the mechanisms behind these AA alterations. METHODS: Twenty AAs, TNF-α, and IL-6 were analyzed in fasting serum samples from a total of 198 individuals (56 drug-naïve patients with T2DM, 69 patients IFG, and 73 healthy controls). The C2C12 mouse myoblast cell lines were used to examine the changes of MAFbx and MuRF1 expressions, which are muscle specific E3 ligases acting as major mediators of skeletal muscle proteolysis, after development of insulin resistance induced by palmitate treatment...
March 27, 2018: Diabetes Research and Clinical Practice
Pan-Pan Tan, Bian-Hua Zhou, Wen-Peng Zhao, Liu-Shu Jia, Jing Liu, Hong-Wei Wang
This study was designed to investigate the mechanisms of excessive fluoride-induced apoptosis via mitochondria-mediated pathway in skeletal muscle cells (C2C12 cells). C2C12 cells were cultured with the fluoride concentrations (0, 1, and 2.5 mmol/L) for 48 h. The morphology and ultrastructural changes of C2C12 cells were observed using a light microscope and transmission electron microscope (TEM). The protein expression levels of apoptosis factors, including Bax, Bcl-2, cytochrome c (Cyt c), caspase-3, and caspase-9, were measured using real-time polymerase chain reaction (real-time PCR) and immunocytofluorescence...
March 29, 2018: Biological Trace Element Research
Lili Zhu, Xiaoyun Zhu, Guibo Sun, Xiangbao Meng, Min Wang, Hanming Cui, Jialong Wang, Yadong Zhai, Ke Yang, Yang Tang, Xiaobo Sun, Ximing Liu
Intricate health problems, such as insulin resistance (IR) and its associated diseases, call for multi-targeted therapies with few side effects. Based on traditional Chinese medicine (TCM), Dai-Zong-Fang (DZF) is an herbal formula mainly composed of Rhizoma Coptidis (Huanglian) and Fructus Aurantii Immaturus (Zhishi), of which berberine and naringin are the main constituents. Though DZF has been clinically used for treatment of IR and metabolic syndrome for decades, its mechanism in vivo remains unknown. In the present study, we observed that both DZF and metformin, the first-line drug for type 2 diabetes, ameliorated insulin resistance with significant improvement of oral glucose tolerance test (OGTT) and homeostasis model assessment of IR (HOMA-IR) level in diabetic C57BL/Ksj-Lepr db -/- ( db/db ) mice...
2018: Frontiers in Physiology
Jiaying Qiu, Qingqing Fang, Tongtong Xu, Changyue Wu, Lai Xu, Lingbin Wang, Xiaoming Yang, Shu Yu, Qi Zhang, Fei Ding, Hualin Sun
Skeletal muscle atrophy occurs under various conditions, such as disuse, denervation, fasting, aging, and various diseases. Although the underlying molecular mechanisms are still not fully understood, skeletal muscle atrophy is closely associated with reactive oxygen species (ROS) overproduction. In this study, we aimed to investigate the involvement of ROS in skeletal muscle atrophy from the perspective of gene regulation, and further examine therapeutic effects of antioxidants on skeletal muscle atrophy. Microarray data showed that the gene expression of many positive regulators for ROS production were up-regulated and the gene expression of many negative regulators for ROS production were down-regulated in mouse soleus muscle atrophied by denervation (sciatic nerve injury)...
2018: Frontiers in Physiology
Béatrice Chabi, Gilles Fouret, Jérome Lecomte, Fabienne Cortade, Laurence Pessemesse, Narjès Baati, Charles Coudray, Ligen Lin, Qiang Tong, Chantal Wrutniak-Cabello, François Casas, Christine Feillet-Coudray
Cardiolipin (CL) is a phospholipid at the heart of mitochondrial metabolism, which plays a key role in mitochondrial function and bioenergetics. Among mitochondrial activity regulators, SIRT3 plays a crucial role in controlling the acetylation status of many enzymes participating in the energy metabolism in particular concerning lipid metabolism and fatty acid oxidation. Data suggest that possible connection may exist between SIRT3 and CL status that has not been evaluated in skeletal muscle. In the present study, we have characterized skeletal muscle lipids as well as mitochondrial lipids composition in mice overexpressing long (SIRT3-M1) and short (SIRT3-M3) isoforms of SIRT3...
March 27, 2018: Journal of Bioenergetics and Biomembranes
Hiroki Tanaka, Katsuhiro Okuda, Seiji Ohtani, Masaru Asari, Kie Horioka, Shotaro Isozaki, Akira Hayakawa, Katsuhiro Ogawa, Shiono Hiroshi, Keiko Shimizu
Electrical injury is damage caused by an electrical current passing through the body. We have previously reported that irregular stripes crossing skeletal muscle fibers (python pattern) and multiple small nuclei arranged in the longitudinal direction of the muscle fibers (chained nuclear change) are uniquely observed by histopathological analysis in the skeletal muscle tissues of patients with electrical injury. However, it remains unclear whether these phenomena are caused by the electrical current itself or by the joule heat generated by the electric current passing through the body...
November 2, 2017: Legal Medicine
Isabel A Hujoel, David H Johnson, Benjamin Lebwohl, Daniel Leffler, Sonia Kupfer, Tsung-Teh Wu, Joseph A Murray, Alberto Rubio-Tapia
BACKGROUND: Whipple disease (WD) is an infection caused by the bacterium Tropheryma whipplei (TW). Few cases have been reported in the USA. AIMS: To report on the demographics, clinical manifestations, diagnostic findings, treatment, and outcomes of TW infection. METHODS: Cases of TW infection diagnosed from 1995 to 2010 were identified in three US referral centers and from 1995 to 2015 in one. Definite classic WD was defined by positive periodic acid-Schiff (PAS) staining and probable WD by specific positive TW polymerase chain reaction (PCR) of intestinal specimens...
March 23, 2018: Digestive Diseases and Sciences
Shurun Zhang, Xiaoling Chen, Zhiqing Huang, Daiwen Chen, Bing Yu, Hong Chen, Junqiu Luo, Jun He, Ping Zheng, Jie Yu
Leucine, one of the branched-chain amino acids, is the only amino acid to regulate protein turnover in skeletal muscle. Leucine not only increases muscle protein synthesis, but also decreases muscle protein degradation. It is well documented that leucine plays a positive role in differentiation of murine muscle cells. However, the role of leucine on porcine myoblast differentiation and its mechanism remains unclear. In this study, porcine myoblasts were induced to differentiate with differentiation medium containing different concentrations of leucine, and wortmannin was used to interdict the activity of protein kinase B (Akt)...
April 2018: British Journal of Nutrition
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