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https://www.readbyqxmd.com/read/28334834/aav-mediated-transfer-of-fkrp-shows-therapeutic-efficacy-in-a-murine-model-but-requires-control-of-gene-expression
#1
Evelyne Gicquel, Natacha Maizonnier, Steven J Foltz, William J Martin, Nathalie Bourg, Fedor Svinartchouk, Karine Charton, Aaron M Beedle, Isabelle Richard
Limb Girdle Muscular Dystrophies type 2I (LGMD2I), a recessive autosomal muscular dystrophy, is caused by mutations in the Fukutin Related Protein (FKRP) gene. It has been proposed that FKRP, a ribitol-5-phosphate transferase, is a participant in α-dystroglycan (αDG) glycosylation, which is important to ensure the cell/matrix anchor of muscle fibers. A LGMD2I knock-in mouse model was generated to express the most frequent mutation (L276I) encountered in patients. The expression of FKRP was not altered neither at transcriptional nor at translational levels, but its function was impacted since abnormal glycosylation of αDG was observed...
March 3, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28329051/caloric-restriction-mimetics-slow-aging-of-neuromuscular-synapses-and-muscle-fibers
#2
Jessica Stockinger, Nicholas Maxwell, Dillon Shapiro, Rafael deCabo, Gregorio Valdez
Resveratrol and metformin have been shown to mimic some aspects of caloric restriction and exercise. However, it remains unknown if these molecules also slow age-related synaptic degeneration, as previously shown for caloric restriction and exercise. In this study, we examined the structural integrity of neuromuscular junctions (NMJs) in 2-year-old mice treated with resveratrol and metformin starting at 1 year of age. We found that resveratrol significantly slows aging of NMJs in the extensor digitorum longus muscle of 2-year-old mice...
March 7, 2017: Journals of Gerontology. Series A, Biological Sciences and Medical Sciences
https://www.readbyqxmd.com/read/28329045/extensive-type-ii-muscle-fiber-atrophy-in-elderly-female-hip-fracture-patients
#3
Irene Fleur Kramer, Tim Snijders, Joey S J Smeets, Marika Leenders, Janneau van Kranenburg, Marcel den Hoed, Lex B Verdijk, Martijn Poeze, Luc J C van Loon
Background: Sarcopenia, or the loss of muscle mass and strength, is known to increase the risk for falls and (hip) fractures in older people. The objective of this study was to assess the skeletal muscle fiber characteristics in elderly female hip fracture patients. Method: Percutaneous needle biopsies were collected from the vastus lateralis muscle in 15 healthy young women (20 ± 0.4 years), 15 healthy elderly women (79 ± 1.7 years), and 15 elderly women with a fall-related hip fracture (82 ± 1...
January 20, 2017: Journals of Gerontology. Series A, Biological Sciences and Medical Sciences
https://www.readbyqxmd.com/read/28326439/a-distinct-functional-distribution-of-%C3%AE-and-%C3%AE-motoneurons-in-the-rat-trigeminal-motor-nucleus
#4
Yukako Morita-Isogai, Hajime Sato, Mitsuru Saito, Eriko Kuramoto, Dong Xu Yin, Takeshi Kaneko, Takashi Yamashiro, Kenji Takada, Seog Bae Oh, Hiroki Toyoda, Youngnam Kang
Gamma-motoneurons (γMNs) play a crucial role in regulating isometric muscle contraction. The slow jaw-closing during mastication is one of the most functional isometric contractions, which is developed by the rank-order recruitment of alpha-motoneurons (αMNs) in a manner that reflects the size distribution of αMNs. In a mouse spinal motor nucleus, there are two populations of small and large MNs; the former was identified as a population of γMNs based on the positive expression of the transcription factor estrogen-related receptor 3 (Err3) and negative expression of the neuronal DNA-binding protein NeuN, and the latter as that of αMNs based on the opposite pattern of immunoreactivity...
March 22, 2017: Brain Structure & Function
https://www.readbyqxmd.com/read/28325000/mechanomyography-signals-in-spastic-muscle-and-the-correlation-with-the-modified-ashworth-scale
#5
E L Santos, M C Santos, E Krueger, G N Nogueira-Neto, P Nohama
The modified Ashworth scale (MAS) is the most widely used measurement technique to assess levels of spasticity. In MAS, the evaluator graduates spasticity considering his/her subjective analysis of the muscular endurance during passive stretching. Therefore, it is a subjective scale. Mechanomyography (MMG) allows registering the vibrations generated by muscle contraction and stretching events that propagate through the tissue until the surface of the skin. With this in mind, this study aimed to investigate possible correlations between MMG signal and muscle spasticity levels determined by MAS...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28323991/whole-body-vibration-mimics-the-metabolic-effects-of-exercise-in-male-leptin-receptor-deficient-mice
#6
Meghan E McGee-Lawrence, Karl H Wenger, Sudipta Misra, Catherine L Davis, Norman K Pollock, Mohammed Elsalanty, Kehong Ding, Carlos M Isales, Mark W Hamrick, Joanna R Erion, Marlena Wosiski-Kuhn, Phonepasong Arounleut, Mark P Mattson, Roy G Cutler, Jack C Yu, Alexis M Stranahan
Whole-body vibration has gained attention as a potential exercise mimetic, but direct comparisons with the metabolic effects of exercise are scarce. To determine whether whole-body vibration recapitulates the metabolic and osteogenic effects of physical activity, we exposed male wildtype (Wt) and leptin receptor deficient (db/db) mice to daily treadmill exercise or whole-body vibration for three months. Body weights were analyzed and compared with Wt and db/db mice that remained sedentary. Glucose and insulin tolerance testing revealed comparable attenuation of hyperglycemia and insulin resistance in db/db mice following treadmill exercise or whole-body vibration...
March 15, 2017: Endocrinology
https://www.readbyqxmd.com/read/28320672/spatial-analysis-of-multichannel-surface-emg-in-hemiplegic-stroke
#7
Ghulam Rasool, Babak Afsharipour, Nina Suresh, William Z Rymer
We investigated spatial activation patterns of upper extremity muscles during isometric force generation in both intact persons and in hemispheric stroke survivors. We used a 128-channel surface electromyogram (EMG) grid to record the electrical activity of biceps brachii muscles during these contractions. EMG data were processed to develop 2-dimensional root mean square (RMS) maps of muscle activity. Our objective was to determine whether motor impairments following stroke were associated with changes in the muscle activity maps and in the spatial distribution of muscular activation...
March 15, 2017: IEEE Transactions on Neural Systems and Rehabilitation Engineering
https://www.readbyqxmd.com/read/28317484/redox-systems-antioxidants-and-sarcopenia
#8
Bertrand Fougère, Gabor Abellan van Kan, Bruno Vellas, Matteo Cesari
According to the free-radical theory of aging, oxidative stress is a key contributor to the onset of age-related conditions by inducing structural and functional alterations in several cellular components. Although innate defenses exist for limiting the occurrence of such detrimental effects, their ability to counteract the continuous and large production of reactive oxygen species becomes increasingly inefficient with aging. In this context, interventions aimed at preserving the homeostatic balance between oxidant production and antioxidant protection may be beneficial on the pathophysiological modifications and clinical manifestations featuring the aging process...
March 17, 2017: Current Protein & Peptide Science
https://www.readbyqxmd.com/read/28315384/the-absorption-enhancer-sodium-deoxycholate-promotes-high-gene-transfer-in-skeletal-muscles
#9
Christian Leborgne, Debborah Alimi-Guez, Nelly El Shafey, Laetitia van Wittenberghe, Pascal Bigey, Daniel Scherman, Antoine Kichler
Gene delivery to skeletal muscle is a promising strategy for the treatment of muscle disorders and for the systemic secretion of therapeutic proteins. In addition, muscle is an attractive target tissue because it is easily accessible. However, very few synthetic vectors proved capable of surpassing naked DNA mediated muscle gene transfer. In fact, only neutral copolymers, in particular poloxamers, demonstrated capacities to increase transgene expression in skeletal muscles. Here, we studied in vitro and in vivo behaviour of different bile salts...
March 14, 2017: International Journal of Pharmaceutics
https://www.readbyqxmd.com/read/28304076/sexual-dimorphism-in-laryngeal-muscle-fibers-and-ultrasonic-vocalizations-in-the-adult-rat
#10
Charles Lenell, Aaron M Johnson
OBJECTIVE: The human voice is sexually dimorphic in obvious ways, such as differences in fundamental frequency and gross laryngeal anatomy, but also in less apparent ways, such as in the prevalence and types of voice disorders and the manifestation of voice changes in advanced age. Differences between males and females are rarely explored, however, in mechanistic animal studies. The goal of this study was to explore sexual dimorphism in laryngeal function and structure in adult rats by examining ultrasonic vocalization acoustics and muscle fiber size and type in the thyroarytenoid muscle...
March 17, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28301525/muscle-activities-during-walking-and-running-at-energetically-optimal-transition-speed-under-normobaric-hypoxia-on-gradient-slopes
#11
Daijiro Abe, Yoshiyuki Fukuoka, Masahiro Horiuchi
Energy cost of transport per unit distance (CoT; J·kg-1·km-1) displays a U-shaped fashion in walking and a linear fashion in running as a function of gait speed (v; km·h-1). There exists an intersection between U-shaped and linear CoT-v relationships, being termed energetically optimal transition speed (EOTS; km·h-1). Combined effects of gradient and moderate normobaric hypoxia (15.0% O2) were investigated when walking and running at the EOTS in fifteen young males. The CoT values were determined at eight walking speeds (2...
2017: PloS One
https://www.readbyqxmd.com/read/28292879/%C3%AE-gpa-treatment-leads-to-elevated-basal-metabolic-rate-and-enhanced-hypoxic-exercise-tolerance-in-mice
#12
Trenton T Ross, Jeffrey D Overton, Katelyn F Houmard, Stephen T Kinsey
Treatments that increase basal metabolic rate (BMR) and enhance exercise capacity may be useful therapeutic approaches for treating conditions such as type 2 diabetes, obesity, and associated circulatory problems. β-guanidinopropionic acid (β-GPA) supplementation decreases high-energy phosphate concentrations, such as ATP and phosphocreatine (PCr) resulting in an energetic challenge that is similar to both exercise programs and hypoxic conditions. In this study, we administered β-GPA to mice for 2 or 6 weeks, and investigated the effect on muscle energetic status, body and muscle mass, muscle capillarity, BMR, and normoxic and hypoxic exercise tolerance (NET and HET, respectively)...
March 2017: Physiological Reports
https://www.readbyqxmd.com/read/28292875/in-vivo-ca-2-dynamics-induced-by-ca-2-injection-in-individual-rat-skeletal-muscle-fibers
#13
Mario Wakizaka, Hiroaki Eshima, Yoshinori Tanaka, Hideki Shirakawa, David C Poole, Yutaka Kano
In contrast to cardiomyocytes, store overload-induced calcium ion (Ca(2+)) release (SOICR) is not considered to constitute a primary Ca(2+) releasing system from the sarcoplasmic reticulum (SR) in skeletal muscle myocytes. In the latter, voltage-induced Ca(2+) release (VICR) is regarded as the dominant mechanism facilitating contractions. Any role of the SOICR in the regulation of cytoplasmic Ca(2+) concentration ([Ca(2+)]i) and its dynamics in skeletal muscle in vivo remains poorly understood. By means of in vivo single fiber Ca(2+) microinjections combined with bioimaging techniques, we tested the hypothesis that the [Ca(2+)]i dynamics following Ca(2+) injection would be amplified and fiber contraction facilitated by SOICR...
March 2017: Physiological Reports
https://www.readbyqxmd.com/read/28291304/mitochondrial-dysfunction-in-calf-muscles-of-patients-with-combined-peripheral-arterial-disease-and-diabetes-type-2
#14
Brian Lindegaard Pedersen, Niels Bækgaard, Bjørn Quistorff
BACKGROUND: This study elucidate the effects on muscle mitochondrial function in patients suffering from combined peripheral arterial disease (PAD) and type 2 diabetes (T2D) and the relation to patient symptoms and treatment. METHODS: Near Infra Red Spectroscopy (NIRS) calf muscle exercise tests were conducted on Forty subjects, 15 (PAD), 15 (PAD+T2D) and 10 healthy age matched controls (CTRL) recruited from the vascular outpatient clinic at Gentofte County Hospital, Denmark...
March 14, 2017: International Angiology: a Journal of the International Union of Angiology
https://www.readbyqxmd.com/read/28290154/the-role-of-calpains-in-ventilator-induced-diaphragm-atrophy
#15
Xiaoping Zhu, Hieronymus W H van Hees, Leo Heunks, Feifei Wang, Lei Shao, Jiaru Huang, Lei Shi, Shaolin Ma
BACKGROUND: Controlled mechanical ventilation (CMV) is associated with diaphragm dysfunction. Dysfunction results from muscle atrophy and injury of diaphragm muscle fibers. Enhanced proteolysis and reduced protein synthesis play an important role in the development of atrophy. The current study is to evaluate the effects of the calpains inhibitor calpeptin on the development of diaphragm atrophy and activation of key enzymes of the ubiquitin-proteasome pathway in rats under CMV. METHODS: Three groups of rats were studied: control animals (CON, n = 8), rats subjected to 24 h of MV (CMV, n = 8), and rats subjected to 24 h of MV after administration of the calpain inhibitor calpeptin (CMVC, n = 8)...
December 2017: Intensive Care Medicine Experimental
https://www.readbyqxmd.com/read/28289716/muscular-dystrophy-in-ptfr-cavin-1-null-mice
#16
Shi-Ying Ding, Libin Liu, Paul F Pilch
ice and humans lacking the caveolae component polymerase I transcription release factor (PTRF, also known as cavin-1) exhibit lipo- and muscular dystrophy. Here we describe the molecular features underlying the muscle phenotype for PTRF/cavin-1 null mice. These animals had a decreased ability to exercise, and exhibited muscle hypertrophy with increased muscle fiber size and muscle mass due, in part, to constitutive activation of the Akt pathway. Their muscles were fibrotic and exhibited impaired membrane integrity accompanied by an apparent compensatory activation of the dystrophin-glycoprotein complex along with elevated expression of proteins involved in muscle repair function...
March 9, 2017: JCI Insight
https://www.readbyqxmd.com/read/28289526/aerobic-vs-anaerobic-exercise-training-effects-on-the-cardiovascular-system
#17
REVIEW
Harsh Patel, Hassan Alkhawam, Raef Madanieh, Niel Shah, Constantine E Kosmas, Timothy J Vittorio
Physical exercise is one of the most effective methods to help prevent cardiovascular (CV) disease and to promote CV health. Aerobic and anaerobic exercises are two types of exercise that differ based on the intensity, interval and types of muscle fibers incorporated. In this article, we aim to further elaborate on these two categories of physical exercise and to help decipher which provides the most effective means of promoting CV health.
February 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28288352/effects-of-dechlorane-plus-exposure-on-axonal-growth-musculature-and-motor-behavior-in-embryo-larval-zebrafish
#18
Xiangping Chen, Qiaoxiang Dong, Yuanhong Chen, Zhenxuan Zhang, Changjiang Huang, Yaxian Zhu, Yong Zhang
Developmental neurobehavioral toxicity of Dechlorane Plus (DP) was investigated using the embryo-larval stages of zebrafish (Danio rerio). Normal fertilized embryos were waterborne exposed to DP at 15, 30, 60 μg/L beginning from 6 h post-fertilization (hpf). Larval teratology, motor activity, motoneuron axonal growth and muscle morphology were assessed at different developmental stages. Results showed that DP exposure significantly altered embryonic spontaneous movement, reduced touch-induced movement and free-swimming speed and decreased swimming speed of larvae in response to dark stimulation...
March 10, 2017: Environmental Pollution
https://www.readbyqxmd.com/read/28287512/preparation-and-culture-of-myogenic-precursor-cells-primary-myoblasts-from-skeletal-muscle-of-adult-and-aged-humans
#19
Ana Soriano-Arroquia, Peter D Clegg, Andrew P Molloy, Katarzyna Goljanek-Whysall
Skeletal muscle homeostasis depends on muscle growth (hypertrophy), atrophy and regeneration. During ageing and in several diseases, muscle wasting occurs. Loss of muscle mass and function is associated with muscle fiber type atrophy, fiber type switching, defective muscle regeneration associated with dysfunction of satellite cells, muscle stem cells, and other pathophysiological processes. These changes are associated with changes in intracellular as well as local and systemic niches. In addition to most commonly used rodent models of muscle ageing, there is a need to study muscle homeostasis and wasting using human models, which due to ethical implications, consist predominantly of in vitro cultures...
February 16, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28286533/chinese-herbal-medicine-baoyuan-jiedu-decoction-inhibited-muscle-atrophy-of-cancer-cachexia-through-atrogin-l-and-murf-1
#20
YaNan Zhang, XiaoChun Han, Bing Ouyang, ZhiChun Wu, HuaYun Yu, Yuan Wang, GuoWei Liu, XuMing Ji
The aim of this study is to investigate the mechanisms of Chinese herbal medicine called "Baoyuan Jiedu" (BYJD for short) decoction, improving life quality and preventing muscle atrophy of cancer cachexia model mice. We showed that the effect of BYJD decoction increased body weights of mice and reduced tumor volume and tumor mass. Furthermore, BYJD decoction increased the gastrocnemii mass and the transverse diameter of muscle fiber morphology. Moreover, BYJD reduced the expression of Atrogin-1 and MuRF-1 protein...
2017: Evidence-based Complementary and Alternative Medicine: ECAM
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