Human laminin-111 and laminin-211 protein therapy prevents muscle disease progression in an immunodeficient mouse model of LAMA2-CMD
Laminin- α2-related congenital muscular dystrophy (LAMA2-CMD) is a devastating genetic disease caused by mutations in the LAMA2 gene. These mutations result in progressive muscle wasting and inflammation leadin... (Source: Skeletal Muscle)
Source: Skeletal Muscle - June 4, 2020 Category: Research Authors: Pamela Barraza-Flores, Hailey J. Hermann, Christina R. Bates, Tyler G. Allen, Timothy T. Grunert and Dean J. Burkin Tags: Research Source Type: research
Pluripotent stem cell-derived skeletal muscle fibers preferentially express myosin heavy-chain isoforms associated with slow and oxidative muscles
Skeletal muscle function is essential for health, and it depends on the proper activity of myofibers and their innervating motor neurons. Each adult muscle is composed of different types of myofibers with dist... (Source: Skeletal Muscle)
Source: Skeletal Muscle - June 3, 2020 Category: Research Authors: Tania Incitti, Alessandro Magli, Asher Jenkins, Karena Lin, Ami Yamamoto and Rita C. R. Perlingeiro Tags: Research Source Type: research
SMN-deficiency disrupts SERCA2 expression and intracellular Ca2+ signaling in cardiomyocytes from SMA mice and patient-derived iPSCs
Spinal muscular atrophy (SMA) is a neurodegenerative disease characterized by loss of alpha motor neurons and skeletal muscle atrophy. The disease is caused by mutations of the SMN1 gene that result in reduced fu... (Source: Skeletal Muscle)
Source: Skeletal Muscle - May 8, 2020 Category: Research Authors: Guzal Khayrullina, Kasey E. Moritz, James F. Schooley, Naheed Fatima, Coralie Viollet, Nikki M. McCormack, Jeremy T. Smyth, Martin L. Doughty, Clifton L. Dalgard, Thomas P. Flagg and Barrington G. Burnett Tags: Research Source Type: research
Exclusive vital labeling of myonuclei for studying myonuclear arrangement in mouse skeletal muscle tissue
The arrangement of myonuclei in skeletal muscle tissue has long been used as a biomarker for muscle health, but there is a dearth of in vivo exploration of potential effects of myonuclear organization on the f... (Source: Skeletal Muscle)
Source: Skeletal Muscle - May 7, 2020 Category: Research Authors: Robert Louis Hastings, Ryan T. Massopust, Seth G. Haddix, Young il Lee and Wesley J. Thompson Tags: Methodology Source Type: research
Functional effects of muscle PGC-1alpha in aged animals
PGC-1 (peroxisome-proliferator-activated receptor- γ coactivator-1) alpha is a potent transcriptional coactivator that coordinates the activation of numerous metabolic processes. Exercise strongly induces PGC-1... (Source: Skeletal Muscle)
Source: Skeletal Muscle - May 6, 2020 Category: Research Authors: Steven Yang, Emanuele Loro, Shogo Wada, Boa Kim, Wei-Ju Tseng, Kristina Li, Tejvir S. Khurana and Zoltan Arany Tags: Research Source Type: research
Acute conversion of patient-derived Duchenne muscular dystrophy iPSC into myotubes reveals constitutive and inducible over-activation of TGF β-dependent pro-fibrotic signaling
In Duchenne muscular dystrophy (DMD), DYSTROPHIN deficiency exposes myofibers to repeated cycles of contraction/degeneration, ultimately leading to muscle loss and replacement by fibrotic tissue. DMD pathology... (Source: Skeletal Muscle)
Source: Skeletal Muscle - May 2, 2020 Category: Research Authors: Luca Caputo, Alice Granados, Jessica Lenzi, Alessandro Rosa, Slimane Ait-Si-Ali, Pier Lorenzo Puri and Sonia Albini Tags: Research Source Type: research
Expression of MuRF1 or MuRF2 is essential for the induction of skeletal muscle atrophy and dysfunction in a murine pulmonary hypertension model
Pulmonary hypertension leads to right ventricular heart failure and ultimately to cardiac cachexia. Cardiac cachexia induces skeletal muscles atrophy and contractile dysfunction. MAFbx and MuRF1 are two key pr... (Source: Skeletal Muscle)
Source: Skeletal Muscle - April 27, 2020 Category: Research Authors: Thanh Nguyen, T. Scott Bowen, Antje Augstein, Antje Schauer, Alexander Gasch, Axel Linke, Siegfried Labeit and Volker Adams Tags: Research Source Type: research
Angiotensin-converting-enzyme inhibitor prevents skeletal muscle fibrosis in myocardial infarction mice
Transforming growth factor beta (TGF- β)-Smad2/3 is the major signaling pathway of fibrosis, which is characterized by the excessive production and accumulation of extracellular matrix (ECM) components, includi... (Source: Skeletal Muscle)
Source: Skeletal Muscle - April 25, 2020 Category: Research Authors: Naoya Kakutani, Shingo Takada, Hideo Nambu, Junichi Matsumoto, Takaaki Furihata, Takashi Yokota, Arata Fukushima and Shintaro Kinugawa Tags: Research Source Type: research
Efficient engraftment of pluripotent stem cell-derived myogenic progenitors in a novel immunodeficient mouse model of limb girdle muscular dystrophy 2I
Defects in α-dystroglycan (DG) glycosylation characterize a group of muscular dystrophies known as dystroglycanopathies. One of the key effectors in the α-DG glycosylation pathway is the glycosyltransferase fu... (Source: Skeletal Muscle)
Source: Skeletal Muscle - April 22, 2020 Category: Research Authors: Karim Azzag, Carolina Ortiz-Cordero, Nelio A. J. Oliveira, Alessandro Magli, Sridhar Selvaraj, Sudheer Tungtur, Weston Upchurch, Paul A. Iaizzo, Qi Long Lu and Rita C. R. Perlingeiro Tags: Research Source Type: research
Correction to: Expressing a Z-disk nebulin fragment innebulin-deficient mouse muscle: effects on muscle structure and function
Following the publication of this paper [1], it was brought to the authors ’ attention that one of the contributing authors was left off of the paper. The authors apologize for the unfortunate oversight. In thi... (Source: Skeletal Muscle)
Source: Skeletal Muscle - April 20, 2020 Category: Research Authors: Frank Li, Justin Kolb, Julie Crudele, Paola Tonino, Zaynab Hourani, John E. Smith III, Jeffrey S. Chamberlain and Henk Granzier Tags: Correction Source Type: research
Transgenic mice expressing tunable levels of DUX4 develop characteristic facioscapulohumeral muscular dystrophy-like pathophysiology ranging in severity
All types of facioscapulohumeral muscular dystrophy (FSHD) are caused by the aberrant activation of the somatically silent DUX4 gene, the expression of which initiates a cascade of cellular events ultimately lead... (Source: Skeletal Muscle)
Source: Skeletal Muscle - April 11, 2020 Category: Research Authors: Takako I. Jones, Guo-Liang Chew, Pamela Barraza-Flores, Spencer Schreier, Monique Ramirez, Ryan D. Wuebbles, Dean J. Burkin, Robert K. Bradley and Peter L. Jones Tags: Research Source Type: research
High-throughput proteomics fiber typing (ProFiT) for comprehensive characterization of single skeletal muscle fibers
Skeletal muscles are composed of a heterogeneous collection of fiber types with different physiological adaption in response to a stimulus and disease-related conditions. Each fiber has a specific molecular ex... (Source: Skeletal Muscle)
Source: Skeletal Muscle - March 23, 2020 Category: Research Authors: Sebastian Kallabis, Lena Abraham, Stefan M üller, Verena Dzialas, Clara Türk, Janica Lea Wiederstein, Theresa Bock, Hendrik Nolte, Leonardo Nogara, Bert Blaauw, Thomas Braun and Marcus Krüger Tags: Methodology Source Type: research
A novel transplantable model of lung cancer-associated tissue loss and disrupted muscle regeneration
Cancer-associated muscle wasting (CAW), a symptom of cancer cachexia, is associated with approximately 20% of lung cancer deaths and remains poorly characterized on a mechanistic level. Current animal models f... (Source: Skeletal Muscle)
Source: Skeletal Muscle - March 9, 2020 Category: Research Authors: Paige C. Arneson-Wissink, Alexandra M. Ducharme and Jason D. Doles Tags: Research Source Type: research
Induction of the nicotinamide riboside kinase NAD+ salvage pathway in a model of sarcoplasmic reticulum dysfunction
Hexose-6-Phosphate Dehydrogenase (H6PD) is a generator of NADPH in the Endoplasmic/Sarcoplasmic Reticulum (ER/SR). Interaction of H6PD with 11 β-hydroxysteroid dehydrogenase type 1 provides NADPH to support oxo... (Source: Skeletal Muscle)
Source: Skeletal Muscle - February 19, 2020 Category: Research Authors: Craig L. Doig, Agnieszka E. Zielinska, Rachel S. Fletcher, Lucy A. Oakey, Yasir S. Elhassan, Antje Garten, David Cartwright, Silke Heising, Ahmed Alsheri, David G. Watson, Cornelia Prehn, Jerzy Adamski, Daniel A. Tennant and Gareth G. Lavery Tags: Research Source Type: research
Skeletal muscle as an experimental model of choice to study tissue aging and rejuvenation
Skeletal muscle is among the most age-sensitive tissues in mammal organisms. Significant changes in its resident stem cells (i.e., satellite cells, SCs), differentiated cells (i.e., myofibers), and extracellul... (Source: Skeletal Muscle)
Source: Skeletal Muscle - February 7, 2020 Category: Research Authors: Jessy Etienne, Chao Liu, Colin M. Skinner, Michael J. Conboy and Irina M. Conboy Tags: Review Source Type: research
