Attenuated Ca2+ release in a mouse model of limb girdle muscular dystrophy 2A
Mutations in CAPN3 cause limb girdle muscular dystrophy type 2A (LGMD2A), a progressive muscle wasting disease. CAPN3 is a non-lysosomal, Ca-dependent, muscle-specific proteinase. Ablation of CAPN3 (calpain-3 kno... (Source: Skeletal Muscle)
Source: Skeletal Muscle - February 24, 2016 Category: Research Authors: Marino DiFranco, Irina Kramerova, Julio L. Vergara and Melissa Jan Spencer Source Type: research
Prenatal muscle development in a mouse model for the secondary dystroglycanopathies
The defective glycosylation of α-dystroglycan is associated with a group of muscular dystrophies that are collectively referred to as the secondary dystroglycanopathies. Mutations in the gene encoding fukutin-... (Source: Skeletal Muscle)
Source: Skeletal Muscle - February 19, 2016 Category: Research Authors: Jihee Kim, Mark Hopkinson, Manoli Kavishwar, Marta Fernandez-Fuente and Susan Carol Brown Source Type: research
Failed upregulation of TFAM protein and mitochondrial DNA in oxidatively deficient fibers of chronic obstructive pulmonary disease locomotor muscle
Low mitochondrial content and oxidative capacity are well-established features of locomotor muscle dysfunction, a prevalent and debilitating systemic occurrence in patients with chronic obstructive pulmonary d... (Source: Skeletal Muscle)
Source: Skeletal Muscle - February 18, 2016 Category: Research Authors: Yana Konokhova, Sally Spendiff, R. Thomas Jagoe, Sudhakar Aare, Sophia Kapchinsky, Norah J. MacMillan, Paul Rozakis, Martin Picard, Mylène Aubertin-Leheudre, Charlotte H. Pion, Jean Bourbeau, Russell T. Hepple and Tanja Taivassalo Source Type: research
Alternating bipolar field stimulation identifies muscle fibers with defective excitability but maintained local Ca2+ signals and contraction
Most cultured enzymatically dissociated adult myofibers exhibit spatially uniform (UNI) contractile responses and Ca2+ transients over the entire myofiber in response to electric field stimuli of either polarity ... (Source: Skeletal Muscle)
Source: Skeletal Muscle - February 5, 2016 Category: Research Authors: Erick O. Hernández-Ochoa, Camilo Vanegas, Shama R. Iyer, Richard M. Lovering and Martin F. Schneider Source Type: research
Exercise-induced mitochondrial p53 repairs mtDNA mutations in mutator mice
Human genetic disorders and transgenic mouse models have shown that mitochondrial DNA (mtDNA) mutations and telomere dysfunction instigate the aging process. Epidemiologically, exercise is associated with grea... (Source: Skeletal Muscle)
Source: Skeletal Muscle - January 31, 2016 Category: Research Authors: Adeel Safdar, Konstantin Khrapko, James M. Flynn, Ayesha Saleem, Michael De Lisio, Adam P. W. Johnston, Yevgenya Kratysberg, Imtiaz A. Samjoo, Yu Kitaoka, Daniel I. Ogborn, Jonathan P. Little, Sandeep Raha, Gianni Parise, Mahmood Akhtar, Bart P. Hettinga, Source Type: research
DNA-binding sequence specificity of DUX4
Misexpression of the double homeodomain transcription factor DUX4 results in facioscapulohumeral muscular dystrophy (FSHD). A DNA-binding consensus with two tandem TAAT motifs based on chromatin IP peaks has b... (Source: Skeletal Muscle)
Source: Skeletal Muscle - January 28, 2016 Category: Research Authors: Yu Zhang, John K. Lee, Erik A. Toso, Joslynn S. Lee, Si Ho Choi, Matthew Slattery, Hideki Aihara and Michael Kyba Source Type: research
Decrease of myofiber branching via muscle-specific expression of the olfactory receptor mOR23 in dystrophic muscle leads to protection against mechanical stress
Abnormal branched myofibers within skeletal muscles are commonly found in diverse animal models of muscular dystrophy as well as in patients. Branched myofibers from dystrophic mice are more susceptible to bre... (Source: Skeletal Muscle)
Source: Skeletal Muscle - January 21, 2016 Category: Research Authors: Christophe Pichavant, Thomas J. Burkholder and Grace K. Pavlath Source Type: research
The ins and outs of muscle stem cell aging
Skeletal muscle has a remarkable capacity to regenerate by virtue of its resident stem cells (satellite cells). This capacity declines with aging, although whether this is due to extrinsic changes in the envir... (Source: Skeletal Muscle)
Source: Skeletal Muscle - January 18, 2016 Category: Research Authors: Andrew S. Brack and Pura Muñoz-Cánoves Source Type: research
Genetic disruption of Ano5 in mice does not recapitulate human ANO5-deficient muscular dystrophy
Anoctamin 5 (ANO5) is a member of a conserved gene family (TMEM16), which codes for proteins predicted to have eight transmembrane domains and putative Ca2+-activated chloride channel (CaCC) activity. It was rece... (Source: Skeletal Muscle)
Source: Skeletal Muscle - December 21, 2015 Category: Research Authors: Jing Xu, Mona El Refaey, Li Xu, Lixia Zhao, Yandi Gao, Kyle Floyd, Tallib Karaze, Paul M. L. Janssen and Renzhi Han Source Type: research
Pervasive satellite cell contribution to uninjured adult muscle fibers
Adult skeletal muscle adapts to functional needs, maintaining consistent numbers of myonuclei and stem cells. Although resident muscle stem cells or satellite cells are required for muscle growth and repair, i... (Source: Skeletal Muscle)
Source: Skeletal Muscle - December 14, 2015 Category: Research Authors: Bradley Pawlikowski, Crystal Pulliam, Nicole Dalla Betta, Gabrielle Kardon and Bradley B. Olwin Source Type: research
CD49d is a disease progression biomarker and a potential target for immunotherapy in Duchenne muscular dystrophy
Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene. The immune inflammatory response also contributes to disease progression in DMD patients. In a previous study, we demonstrated h... (Source: Skeletal Muscle)
Source: Skeletal Muscle - December 10, 2015 Category: Research Authors: Fernanda Pinto-Mariz, Luciana Rodrigues Carvalho, Alexandra Prufer De Queiroz Campos Araujo, Wallace De Mello, Márcia Gonçalves Ribeiro, Maria Do Carmo Soares Alves Cunha, Pedro Hernan Cabello, Ingo Riederer, Elisa Negroni, Isabelle Desguerre, Mariana V Source Type: research
Investigating mechanisms underpinning the detrimental impact of a high-fat diet in the developing and adult hypermuscular myostatin null mouse
Obese adults are prone to develop metabolic and cardiovascular diseases. Furthermore, over-weight expectant mothers give birth to large babies who also have increased likelihood of developing metabolic and car... (Source: Skeletal Muscle)
Source: Skeletal Muscle - December 7, 2015 Category: Research Authors: Antonios Matsakas, Domenick A. Prosdocimo, Robert Mitchell, Henry Collins-Hooper, Natasa Giallourou, Jonathan R. Swann, Paul Potter, Thomas Epting, Mukesh K. Jain and Ketan Patel Source Type: research
PAX7 is required for patterning the esophageal musculature
The mammalian esophageal musculature is unique in that it makes a transition from smooth to skeletal muscle, with most of this process occurring after birth. In order to better understand the mechanisms that c... (Source: Skeletal Muscle)
Source: Skeletal Muscle - December 3, 2015 Category: Research Authors: Daisuke Chihara, Anthony I. Romer, C. Florian Bentzinger, Michael A. Rudnicki and Robert S. Krauss Source Type: research
Elusive sources of variability of dystrophin rescue by exon skipping
Systemic delivery of anti-sense oligonucleotides to Duchenne muscular dystrophy (DMD) patients to induce de novo dystrophin protein expression in muscle (exon skipping) is a promising therapy. Treatment with P... (Source: Skeletal Muscle)
Source: Skeletal Muscle - December 1, 2015 Category: Research Authors: Maria Candida Vila, Margaret Benny Klimek, James S. Novak, Sree Rayavarapu, Kitipong Uaesoontrachoon, Jessica F. Boehler, Alyson A. Fiorillo, Marshall W. Hogarth, Aiping Zhang, Conner Shaughnessy, Heather Gordish-Dressman, Umar Burki, Volker Straub, Qi Lo Source Type: research
Reduced voluntary running performance is associated with impaired coordination as a result of muscle satellite cell depletion in adult mice
Satellite cells, or muscle stem cells, have been thought to be responsible for all muscle plasticity, but recent studies using genetically modified mouse models that allow for the conditional ablation of satel... (Source: Skeletal Muscle)
Source: Skeletal Muscle - November 16, 2015 Category: Research Authors: Janna R. Jackson, Tyler J. Kirby, Christopher S. Fry, Robin L. Cooper, John J. McCarthy, Charlotte A. Peterson and Esther E. Dupont-Versteegden Source Type: research
