Articles

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...

Authors: Frank Li, Justin Kolb, Julie Crudele, Paola Tonino, Zaynab Hourani, John E. Smith III, Jeffrey S. Chamberlain and Henk Granzier

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...

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

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...

Authors: Sebastian Kallabis, Lena Abraham, Stefan Müller, Verena Dzialas, Clara Türk, Janica Lea Wiederstein, Theresa Bock, Hendrik Nolte, Leonardo Nogara, Bert Blaauw, Thomas Braun and Marcus Krüger

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...

Authors: Paige C. Arneson-Wissink, Alexandra M. Ducharme and Jason D. Doles

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 11β-hydroxysteroid dehydrogenase type 1 provides NADPH to support oxo...

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

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...

Authors: Jessy Etienne, Chao Liu, Colin M. Skinner, Michael J. Conboy and Irina M. Conboy

Mechanical factors tune the sensitivity of mdx muscle to eccentric strength loss and its protection by antioxidant and calcium modulators

Dystrophin deficiency sensitizes skeletal muscle of mice to eccentric contraction (ECC)-induced strength loss. ECC protocols distinguish dystrophin-deficient from healthy, wild type muscle, and test the effica...

Authors: Angus Lindsay, Cory W. Baumann, Robyn T. Rebbeck, Samantha L. Yuen, William M. Southern, James S. Hodges, Razvan L. Cornea, David D. Thomas, James M. Ervasti and Dawn A. Lowe

Expressing a Z-disk nebulin fragment in nebulin-deficient mouse muscle: effects on muscle structure and function

Nebulin is a critical thin filament-binding protein that spans from the Z-disk of the skeletal muscle sarcomere to near the pointed end of the thin filament. Its massive size and actin-binding property allows ...

Authors: Frank Li, Justin Kolb, Julie Crudele, Paola Tonino, Zaynab Hourani, John E. Smith III, Jeffrey S. Chamberlain and Henk Granzier

The ERG1a potassium channel increases basal intracellular calcium concentration and calpain activity in skeletal muscle cells

Skeletal muscle atrophy is the net loss of muscle mass that results from an imbalance in protein synthesis and protein degradation. It occurs in response to several stimuli including disease, injury, starvatio...

Authors: Clayton Whitmore, Evan P.S. Pratt, Luke Anderson, Kevin Bradley, Sawyer M. Latour, Mariam N. Hashmi, Albert K. Urazaev, Rod Weilbaecher, Judith K. Davie, Wen-Horng Wang, Gregory H. Hockerman and Amber L. Pond

Development of a high-throughput screen to identify small molecule enhancers of sarcospan for the treatment of Duchenne muscular dystrophy

Duchenne muscular dystrophy (DMD) is caused by loss of sarcolemma connection to the extracellular matrix. Transgenic overexpression of the transmembrane protein sarcospan (SSPN) in the DMD mdx mouse model signifi...

Authors: Cynthia Shu, Ariana N. Kaxon-Rupp, Judd R. Collado, Robert Damoiseaux and Rachelle H. Crosbie

miR-1/206 downregulates splicing factor Srsf9 to promote C2C12 differentiation

Myogenesis is driven by specific changes in the transcriptome that occur during the different stages of muscle differentiation. In addition to controlled transcriptional transitions, several other post-transcr...

Authors: Kristen K. Bjorkman, Massimo Buvoli, Emily K. Pugach, Michael M. Polmear and Leslie A. Leinwand

mTORC2 affects the maintenance of the muscle stem cell pool

The mammalian target of rapamycin complex 2 (mTORC2), containing the essential protein rictor, regulates cellular metabolism and cytoskeletal organization by phosphorylating protein kinases, such as PKB/Akt, P...

Authors: Nathalie Rion, Perrine Castets, Shuo Lin, Leonie Enderle, Judith R. Reinhard and Markus A. Rüegg

In memoriam: Susan Abmayr (1956–2019) – “What do we do? Whatever it takes!”

Authors: Erika R. Geisbrecht and Mary K. Baylies

Two functional variants at 6p21.1 were associated with lean mass

Low lean body mass is the most important predictor of sarcopenia with strong genetic background. The aim of this study was to uncover genetic factors underlying lean mass development.

Authors: Yu-Fang Pei, Wen-Zhu Hu, Xiao-Lin Yang, Xin-Tong Wei, Gui-Juan Feng, Hong Zhang, Hui Shen, Qing Tian, Hong-Wen Deng and Lei Zhang

Barium chloride injures myofibers through calcium-induced proteolysis with fragmentation of motor nerves and microvessels

Local injection of BaCl₂ is an established model of acute injury to study the regeneration of skeletal muscle. However, the mechanism by which BaCl₂ causes muscle injury is unresolved. Because Ba²⁺ inhibits K⁺ ch...

Authors: Aaron B. Morton, Charles E. Norton, Nicole L. Jacobsen, Charmain A. Fernando, D. D. W. Cornelison and Steven S. Segal

LIM and cysteine-rich domains 1 (LMCD1) regulates skeletal muscle hypertrophy, calcium handling, and force

Skeletal muscle mass and strength are crucial determinants of health. Muscle mass loss is associated with weakness, fatigue, and insulin resistance. In fact, it is predicted that controlling muscle atrophy can...

Authors: Duarte M. S. Ferreira, Arthur J. Cheng, Leandro Z. Agudelo, Igor Cervenka, Thomas Chaillou, Jorge C. Correia, Margareta Porsmyr-Palmertz, Manizheh Izadi, Alicia Hansson, Vicente Martínez-Redondo, Paula Valente-Silva, Amanda T. Pettersson-Klein, Jennifer L. Estall, Matthew M. Robinson, K. Sreekumaran Nair, Johanna T. Lanner…

Defective angiogenesis in CXCL12 mutant mice impairs skeletal muscle regeneration

During muscle regeneration, the chemokine CXCL12 (SDF-1) and the synthesis of some specific heparan sulfates (HS) have been shown to be critical. CXCL12 activity has been shown to be heavily influenced by its ...

Authors: David Hardy, Mylène Fefeu, Aurore Besnard, David Briand, Paméla Gasse, Fernando Arenzana-Seisdedos, Pierre Rocheteau and Fabrice Chrétien

Immunohistochemical phenotyping of T cells, granulocytes, and phagocytes in the muscle of cancer patients: association with radiologically defined muscle mass and gene expression

Inflammation is a recognized contributor to muscle wasting. Research in injury and myopathy suggests that interactions between the skeletal muscle and immune cells confer a pro-inflammatory environment that in...

Authors: Ana Anoveros-Barrera, Amritpal S. Bhullar, Cynthia Stretch, Abha R. Dunichand-Hoedl, Karen J. B. Martins, Aja Rieger, David Bigam, Todd McMullen, Oliver F. Bathe, Charles T. Putman, Catherine J. Field, Vickie E. Baracos and Vera C. Mazurak

Ibuprofen inhibited migration of skeletal muscle cells in association with downregulation of p130cas and CrkII expressions

Nonsteroidal anti-inflammatory drugs (NSAIDs) are commonly used to treat sports-related muscle injuries. However, NSAIDs were recently shown to impede the muscle healing process after acute injury. Migration o...

Authors: Chih-Hao Liao, Li-Ping Lin, Tung-Yang Yu, Chih-Chin Hsu, Jong-Hwei S. Pang and Wen-Chung Tsai

miR-146a deficiency does not aggravate muscular dystrophy in mdx mice

Duchenne muscular dystrophy (DMD) is a genetic disease evoked by a mutation in the dystrophin gene. It is associated with progressive muscle degeneration and increased inflammation. Up to this date, mainly ant...

Authors: Iwona Bronisz-Budzyńska, Katarzyna Chwalenia, Olga Mucha, Paulina Podkalicka, Karolina-Bukowska-Strakova, Alicja Józkowicz, Agnieszka Łoboda, Magdalena Kozakowska and Józef Dulak

NAD+ improves neuromuscular development in a zebrafish model of FKRP-associated dystroglycanopathy

Secondary dystroglycanopathies are muscular dystrophies that result from mutations in genes that participate in Dystroglycan glycosylation. Glycosylation of Dystroglycan is essential for muscle fibers to adher...

Authors: Erin C. Bailey, Sarah S. Alrowaished, Elisabeth A. Kilroy, Emma S. Crooks, Daisy M. Drinkert, Chaya M. Karunasiri, Joseph J. Belanger, Andre Khalil, Joshua B. Kelley and Clarissa A. Henry

Estrogen signaling effects on muscle-specific immune responses through controlling the recruitment and function of macrophages and T cells

Estrogen signaling is indispensable for muscle regeneration, yet the role of estrogen in the development of muscle inflammation, especially in the intramuscular T cell response, and the influence on the intrin...

Authors: Zhao Hong Liao, Tao Huang, Jiang Wei Xiao, Rui Cai Gu, Jun Ouyang, Gang Wu and Hua Liao

Regulation of murine skeletal muscle growth by STAT5B is age- and sex-specific

Sexually dimorphic growth has been attributed to the growth hormone (GH)/insulin-like growth factor 1 (IGF1) axis, particularly GH-induced activation of the intracellular signal transducer and activator of tra...

Authors: Ryan G. Paul, Alex S. Hennebry, Marianne S. Elston, John V. Conaglen and Chris D. McMahon

Muscle injury-induced hypoxia alters the proliferation and differentiation potentials of muscle resident stromal cells

Trauma-induced heterotopic ossification (HO) is a complication that develops under three conditions: the presence of an osteogenic progenitor cell, an inducing factor, and a permissive environment. We previous...

Authors: Geneviève Drouin, Vanessa Couture, Marc-Antoine Lauzon, Frédéric Balg, Nathalie Faucheux and Guillaume Grenier

Culturing C2C12 myotubes on micromolded gelatin hydrogels accelerates myotube maturation

Skeletal muscle contributes to roughly 40% of lean body mass, and its loss contributes to morbidity and mortality in a variety of pathogenic conditions. Significant insights into muscle function have been made...

Authors: Lance T. Denes, Lance A. Riley, Joseph R. Mijares, Juan D. Arboleda, Kendra McKee, Karyn A. Esser and Eric T. Wang

A GDF11/myostatin inhibitor, GDF11 propeptide-Fc, increases skeletal muscle mass and improves muscle strength in dystrophic mdx mice

Growth differentiation factor 11 (GDF11) is a member of the transforming growth factor β superfamily. The GDF11 propeptide, which is derived from the GDF11 precursor protein, blocks the activity of GDF11 and i...

Authors: Quan Jin, Chunping Qiao, Jianbin Li, Bin Xiao, Juan Li and Xiao Xiao

Automated image-analysis method for the quantification of fiber morphometry and fiber type population in human skeletal muscle

The quantitative analysis of muscle histomorphometry has been growing in importance in both research and clinical settings. Accurate and stringent assessment of myofibers’ changes in size and number, and alter...

Authors: Perla C. Reyes-Fernandez, Baptiste Periou, Xavier Decrouy, Fréderic Relaix and François Jérôme Authier

Congenital myopathy with hanging big toe due to homozygous myopalladin (MYPN) mutation

Myopalladin (MYPN) is a component of the sarcomere that tethers nebulin in skeletal muscle and nebulette in cardiac muscle to alpha-actinin at the Z lines. Autosomal dominant MYPN mutations cause hypertrophic, di...

Authors: Luciano Merlini, Patrizia Sabatelli, Manuela Antoniel, Valeria Carinci, Fabio Niro, Giuseppe Monetti, Annalaura Torella, Teresa Giugliano, Cesare Faldini and Vincenzo Nigro

Targeted ablation of the cellular inhibitor of apoptosis 1 (cIAP1) attenuates denervation-induced skeletal muscle atrophy

Skeletal muscle atrophy is a pathological condition that contributes to morbidity in a variety of conditions including denervation, cachexia, and aging. Muscle atrophy is characterized as decreased muscle fibe...

Authors: Neena Lala-Tabbert, Rim Lejmi-Mrad, Kristen Timusk, Marina Fukano, Janelle Holbrook, Martine St-Jean, Eric C. LaCasse and Robert G. Korneluk

Preclinical rationale for entinostat in embryonal rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric cancer population. Survival among metastatic RMS patients has remained dismal yet unimproved for years. We previously identified t...

Authors: Narendra Bharathy, Noah E. Berlow, Eric Wang, Jinu Abraham, Teagan P. Settelmeyer, Jody E. Hooper, Matthew N. Svalina, Zia Bajwa, Martin W. Goros, Brian S. Hernandez, Johannes E. Wolff, Ranadip Pal, Angela M. Davies, Arya Ashok, Darnell Bushby, Maria Mancini…

Exogenous expression of the glycosyltransferase LARGE1 restores α-dystroglycan matriglycan and laminin binding in rhabdomyosarcoma

α-Dystroglycan is the highly glycosylated component of the dystrophin-glycoprotein complex (DGC) that binds with high-affinity to extracellular matrix (ECM) proteins containing laminin-G-like (LG) domains via ...

Authors: Daniel Beltrán, Mary E. Anderson, Narendra Bharathy, Teagan P. Settelmeyer, Matthew N. Svalina, Zia Bajwa, John F. Shern, Sakir H. Gultekin, Marco A. Cuellar, Takahiro Yonekawa, Charles Keller and Kevin P. Campbell

Muscle Gene Sets: a versatile methodological aid to functional genomics in the neuromuscular field

The approach of building large collections of gene sets and then systematically testing hypotheses across these collections is a powerful tool in functional genomics, both in the pathway analysis of omics data...

Authors: Apostolos Malatras, Stephanie Duguez and William Duddy

RNA-sequencing reveals altered skeletal muscle contraction, E3 ligases, autophagy, apoptosis, and chaperone expression in patients with critical illness myopathy

Critical illness myopathy (CIM) is associated with severe skeletal muscle wasting and impaired function in intensive care unit (ICU) patients. The mechanisms underlying CIM remain incompletely understood. To e...

Authors: Monica Llano-Diez, Wen Fury, Haruka Okamoto, Yu Bai, Jesper Gromada and Lars Larsson

IMB0901 inhibits muscle atrophy induced by cancer cachexia through MSTN signaling pathway

Cancer cachexia as a metabolic syndrome can lead to at least 25% of cancer deaths. The inhibition of muscle atrophy is a main strategy to treat cancer cachexia. In this process, myostatin (MSTN) can exert a du...

Authors: Dong Liu, Xinran Qiao, Zhijuan Ge, Yue Shang, Yi Li, Wendie Wang, Minghua Chen, Shuyi Si and Shu-zhen Chen

The ancient sarcomeric myosins found in specialized muscles

Striated muscles express an array of sarcomeric myosin motors that are tuned to accomplish specific tasks. Each myosin isoform found in muscle fibers confers unique contractile properties to the fiber in order...

Authors: Lindsey A. Lee, Anastasia Karabina, Lindsey J. Broadwell and Leslie A. Leinwand

Normal inflammation and regeneration of muscle following injury require osteopontin from both muscle and non-muscle cells

Osteopontin is secreted by skeletal muscle myoblasts and macrophages, and its expression is upregulated in muscle following injury. Osteopontin is present in many different structural forms, which vary in thei...

Authors: Dimuthu K. Wasgewatte Wijesinghe, Eleanor J. Mackie and Charles N. Pagel

Late-onset megaconial myopathy in mice lacking group I Paks

Group I Paks are serine/threonine kinases that function as major effectors of the small GTPases Rac1 and Cdc42, and they regulate cytoskeletal dynamics, cell polarity, and transcription. We previously demonstr...

Authors: Giselle A. Joseph, Margaret Hung, Aviva J. Goel, Mingi Hong, Marysia-Kolbe Rieder, Noam D. Beckmann, Madhavika N. Serasinghe, Jerry E. Chipuk, Parvathi M. Devarakonda, David J. Goldhamer, Paulina Aldana-Hernandez, Jonathan Curtis, René L. Jacobs and Robert S. Krauss

Dilated cardiomyopathy-mediated heart failure induces a unique skeletal muscle myopathy with inflammation

Skeletal muscle myopathy and exercise intolerance are diagnostic hallmarks of heart failure (HF). However, the molecular adaptations of skeletal muscles during dilated cardiomyopathy (DCM)-mediated HF are not ...

Authors: Taejeong Song, Palanikumar Manoharan, Douglas P. Millay, Sheryl E. Koch, Jack Rubinstein, Judith A. Heiny and Sakthivel Sadayappan

Metabolic derangements of skeletal muscle from a murine model of glioma cachexia

Cachexia is a complex metabolic disorder and muscle atrophy syndrome, impacting 80% patients with advanced cancers. Malignant glioma is considered to be one of the deadliest human cancers, accounting for about...

Authors: Pengfei Cui, Wei Shao, Caihua Huang, Chang-Jer Wu, Bin Jiang and Donghai Lin

Open-CSAM, a new tool for semi-automated analysis of myofiber cross-sectional area in regenerating adult skeletal muscle

Adult skeletal muscle is capable of complete regeneration after an acute injury. The main parameter studied to assess muscle regeneration efficacy is the cross-sectional area (CSA) of the myofibers as myofiber...

Authors: Thibaut Desgeorges, Sophie Liot, Solene Lyon, Jessica Bouvière, Alix Kemmel, Aurélie Trignol, David Rousseau, Bruno Chapuis, Julien Gondin, Rémi Mounier, Bénédicte Chazaud and Gaëtan Juban

Defects in sarcolemma repair and skeletal muscle function after injury in a mouse model of Niemann-Pick type A/B disease

Niemann-Pick disease type A (NPDA), a disease caused by mutations in acid sphingomyelinase (ASM), involves severe neurodegeneration and early death. Intracellular lipid accumulation and plasma membrane alterat...

Authors: V. Michailowsky, H. Li, B. Mittra, S. R. Iyer, D. A. G. Mazála, M. Corrotte, Y. Wang, E. R. Chin, R. M. Lovering and N. W. Andrews

β-arrestin 1 regulates β2-adrenergic receptor-mediated skeletal muscle hypertrophy and contractility

β₂-adrenergic receptors (β₂ARs) are the target of catecholamines and play fundamental roles in cardiovascular, pulmonary, and skeletal muscle physiology. An important action of β₂AR stimulation on skeletal muscle...

Authors: Jihee Kim, Chad A. Grotegut, James W. Wisler, Tianyu Li, Lan Mao, Minyong Chen, Wei Chen, Paul B. Rosenberg, Howard A. Rockman and Robert J. Lefkowitz

Correction to: HIF prolyl hydroxylase inhibition protects skeletal muscle from eccentric contraction induced injury

Following publication of the original article [1], the authors flagged that there is a discrepancy with the Availability of data and materials statement on page 12 of the article.

Authors: Andrew N. Billin, Samuel E. Honeycutt, Alan V. McDougal, Jaclyn P. Kerr, Zhe Chen, Johannes M. Freudenberg, Deepak K. Rajpal, Guizhen Luo, Henning Fritz Kramer, Robert S. Geske, Frank Fang, Bert Yao, Richard V. Clark, John Lepore, Alex Cobitz, Ram Miller…

The RNA-binding proteins Zfp36l1 and Zfp36l2 act redundantly in myogenesis

Members of the ZFP36 family of RNA-binding proteins regulate gene expression post-transcriptionally by binding to AU-rich elements in the 3’UTR of mRNA and stimulating mRNA degradation. The proteins within thi...

Authors: Hema Bye-A-Jee, Dhamayanthi Pugazhendhi, Samuel Woodhouse, Patrick Brien, Rachel Watson, Martin Turner and Jennifer Pell

Dystrophin R16/17-syntrophin PDZ fusion protein restores sarcolemmal nNOSμ

Loss of sarcolemmal nNOSμ is a common manifestation in a wide variety of muscle diseases and contributes to the dysregulation of multiple muscle activities. Given the critical role sarcolemmal nNOSμ plays in m...

Authors: Aman Patel, Junling Zhao, Yongping Yue, Keqing Zhang, Dongsheng Duan and Yi Lai

HIF prolyl hydroxylase inhibition protects skeletal muscle from eccentric contraction-induced injury

In muscular dystrophy and old age, skeletal muscle repair is compromised leading to fibrosis and fatty tissue accumulation. Therefore, therapies that protect skeletal muscle or enhance repair would be valuable...

Authors: Andrew N. Billin, Samuel E. Honeycutt, Alan V. McDougal, Jaclyn P. Kerr, Zhe Chen, Johannes M. Freudenberg, Deepak K. Rajpal, Guizhen Luo, Henning Fritz Kramer, Robert S. Geske, Frank Fang, Bert Yao, Richard V. Clark, John Lepore, Alex Cobitz, Ram Miller…

Myostatin and activin blockade by engineered follistatin results in hypertrophy and improves dystrophic pathology in mdx mouse more than myostatin blockade alone

Myostatin antagonists are being developed as therapies for Duchenne muscular dystrophy due to their strong hypertrophic effects on skeletal muscle. Engineered follistatin has the potential to combine the hyper...

Authors: Andrea Iskenderian, Nan Liu, Qingwei Deng, Yan Huang, Chuan Shen, Kathleen Palmieri, Robert Crooker, Dianna Lundberg, Niksa Kastrapeli, Brian Pescatore, Alla Romashko, John Dumas, Robert Comeau, Angela Norton, Jing Pan, Haojing Rong…

A novel tetracycline-responsive transgenic mouse strain for skeletal muscle-specific gene expression

The tetracycline-responsive system (Tet-ON/OFF) has proven to be a valuable tool for manipulating gene expression in an inducible, temporal, and tissue-specific manner. The purpose of this study was to create ...

Authors: Masahiro Iwata, Davis A. Englund, Yuan Wen, Cory M. Dungan, Kevin A. Murach, Ivan J. Vechetti Jr, Christopher B. Mobley, Charlotte A. Peterson and John J. McCarthy

Automated muscle histopathology analysis using CellProfiler

Histological assessment of skeletal muscle sections is important for the research of muscle physiology and diseases. Quantifiable measures of skeletal muscle often include mean fiber diameter, fiber size distr...

Authors: Yeh Siang Lau, Li Xu, Yandi Gao and Renzhi Han

Muscle membrane integrity in Duchenne muscular dystrophy: recent advances in copolymer-based muscle membrane stabilizers

The scientific premise, design, and structure-function analysis of chemical-based muscle membrane stabilizing block copolymers are reviewed here for applications in striated muscle membrane injury. Synthetic b...

Authors: Evelyne M. Houang, Yuk Y. Sham, Frank S. Bates and Joseph M. Metzger