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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/28333907/the-effect-of-placing-flow-diverting-stents-in-intracranial-collateral-arteries-of-miniature-pig
#2
Jiwei Wang, Yanan Ding, Qiuxia Wang, Yanan Wang, Shiqing Mu, Lixin Bi, Youxiang Li
BACKGROUND Flow-diverting stent (FDS) has been suggested as an effective intracranial aneurysm treatment. However, the effects of FDS on collateral branches of an aneurysm parent artery still remain unknown. Thus, the present study aimed to comprehensively evaluate the effects of placing a FDS in the intracranial collateral artery, using a miniature pig animal model. MATERIAL AND METHODS Ten healthy miniature pigs were included in the study: one pig was reserved as a control and the remaining nine pigs were placed in three experimental groups: FDS (i...
March 23, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28332479/3d-tissue-formation-by-stacking-detachable-cell-sheets-formed-on-nanofiber-mesh
#3
Min Sung Kim, Byungjun Lee, Hong Nam Kim, Seokyoung Bang, Hee Seok Yang, Seong Min Kang, Kahp-Yang Suh, Suk-Hee Park, Noo Li Jeon
We present a novel approach for assembling 3D tissue by layer-by-layer stacking of cell sheets formed on aligned nanofiber mesh. A rigid frame was used to repeatedly collect aligned electrospun PCL (polycaprolactone) nanofiber to form a mesh structure with average distance between fibers 6.4 µm. When human umbilical vein endothelial cells (HUVECs), human foreskin dermal fibroblasts, and skeletal muscle cells (C2C12) were cultured on the nanofiber mesh, they formed confluent monolayers and could be handled as continuous cell sheets with areas 3 × 3 cm(2) or larger...
March 23, 2017: Biofabrication
https://www.readbyqxmd.com/read/28331320/the-complex-of-pamam-oh-dendrimer-with-angiotensin-1-7-prevented-the-disuse-induced-skeletal-muscle-atrophy-in-mice
#4
Valeria Márquez-Miranda, Johanna Abrigo, Juan Carlos Rivera, Ingrid Araya-Durán, Javier Aravena, Felipe Simon, Nicolás Pacheco, Fernando Danilo González-Nilo, Claudio Cabello-Verrugio
Angiotensin (1-7) (Ang-(1-7)) is a bioactive heptapeptide with a short half-life and has beneficial effects in several tissues - among them, skeletal muscle - by preventing muscle atrophy. Dendrimers are promising vehicles for the protection and transport of numerous bioactive molecules. This work explored the use of a neutral, non-cytotoxic hydroxyl-terminated poly(amidoamine) (PAMAM-OH) dendrimer as an Ang-(1-7) carrier. Bioinformatics analysis showed that the Ang-(1-7)-binding capacity of the dendrimer presented a 2:1 molar ratio...
2017: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/28331075/myosin-activity-drives-actomyosin-bundle-formation-and-organization-in-contractile-cells-of-the-c-elegans-spermatheca
#5
Alison C E Wirshing, Erin J Cram
Stress fibers, contractile actomyosin bundles, are important for cellular force production and adaptation to physical stress and have been well-studied within the context of cell migration. However, less is known about actomyosin bundle formation and organization in vivo and in specialized contractile cells, such as smooth muscle and myoepithelial cells. The Caenorhabditis elegans spermatheca is a bag-like organ of 24 myoepithelial cells that houses the sperm and is the site of fertilization. During ovulation, spermathecal cells are stretched by oocyte entry and then coordinately contract to expel the fertilized embryo into the uterus...
March 22, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/28329051/caloric-restriction-mimetics-slow-aging-of-neuromuscular-synapses-and-muscle-fibers
#6
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
#7
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
#8
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/28326040/effects-of-topical-icing-on-inflammation-angiogenesis-revascularization-and-myofiber-regeneration-in-skeletal-muscle-following-contusion-injury
#9
Daniel P Singh, Zohreh Barani Lonbani, Maria A Woodruff, Tony J Parker, Roland Steck, Jonathan M Peake
Contusion injuries in skeletal muscle commonly occur in contact sport and vehicular and industrial workplace accidents. Icing has traditionally been used to treat such injuries under the premise that it alleviates pain, reduces tissue metabolism, and modifies vascular responses to decrease swelling. Previous research has examined the effects of icing on inflammation and microcirculatory dynamics following muscle injury. However, whether icing influences angiogenesis, collateral vessel growth, or myofiber regeneration remains unknown...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28325000/mechanomyography-signals-in-spastic-muscle-and-the-correlation-with-the-modified-ashworth-scale
#10
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/28324992/age-related-neuromuscular-changes-in-semg-of-m-tibialis-anterior-using-higher-order-statistics-gaussianity-linearity-test
#11
Ariba Siddiqi, Sridhar P Arjunan, Dinesh K Kumar
Age-associated changes in the surface electromyogram (sEMG) of Tibialis Anterior (TA) muscle can be attributable to neuromuscular alterations that precede strength loss. We have used our sEMG model of the Tibialis Anterior to interpret the age-related changes and compared with the experimental sEMG. Eighteen young (20-30 years) and 18 older (60-85 years) performed isometric dorsiflexion at 6 different percentage levels of maximum voluntary contractions (MVC), and their sEMG from the TA muscle was recorded. Six different age-related changes in the neuromuscular system were simulated using the sEMG model at the same MVCs as the experiment...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28324764/enhancing-mitofusin-marf-ameliorates-neuromuscular-dysfunction-in-drosophila-models-of-tdp-43-proteinopathies
#12
Bilal Khalil, Marie-Jeanne Cabirol-Pol, Laetitia Miguel, Alexander J Whitworth, Magalie Lecourtois, Jean-Charles Liévens
Transactive response DNA-binding protein 43 kDa (TDP-43) is considered a major pathological protein in amyotrophic lateral sclerosis and frontotemporal lobar degeneration. The precise mechanisms by which TDP-43 dysregulation leads to toxicity in neurons are not fully understood. Using TDP-43-expressing Drosophila, we examined whether mitochondrial dysfunction is a central determinant in TDP-43 pathogenesis. Expression of human wild-type TDP-43 in Drosophila neurons results in abnormally small mitochondria...
February 27, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28323991/whole-body-vibration-mimics-the-metabolic-effects-of-exercise-in-male-leptin-receptor-deficient-mice
#13
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/28321943/development-of-3d-intramural-and-surface-potentials-in-the-lv-microstructural-basis-of-preferential-transmural-conduction
#14
Bryan J Caldwell, Mark L Trew, Ian J Legrice, Bruce H Smaill
INTRODUCTION: Extracellular potentials measured on the heart surfaces are used to infer events that originate deep within the heart wall. We have reconstructed intramural potentials in three dimensions for the first time, and compare these with epicardial and endocardial surface potentials and cardiac microstructure. METHODS AND RESULTS: Extracellular potentials from intramural point stimulation were measured from a high density 3D electrode array in the in vivo pig LV...
March 21, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/28320672/spatial-analysis-of-multichannel-surface-emg-in-hemiplegic-stroke
#15
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/28319875/electrophysiologic-features-of-ulnar-neuropathy-in-childhood-and-adolescence
#16
Ioannis Karakis, Wendy Liew, Heather Szelag Fournier, H Royden Jones, Basil T Darras, Peter B Kang
OBJECTIVE: To analyze patterns of nerve injury in pediatric ulnar neuropathy (PUN). METHODS: Retrospective analysis of 49 children with PUN. RESULTS: Sensory loss in digit V was the prevailing complaint (89%). Predominant localization was at the elbow (55%). Diminished ulnar SNAP was the most common abnormality (71%) with median axon loss estimate (MAXE) of 62%. Dorsal ulnar cutaneous (DUC) sensory nerve action potential (SNAP) was reduced in 55% with MAXE of 43%...
February 20, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28317484/redox-systems-antioxidants-and-sarcopenia
#17
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/28316572/eccentric-ergometer-training-promotes-locomotor-muscle-strength-but-not-mitochondrial-adaptation-in-patients-with-severe-chronic-obstructive-pulmonary-disease
#18
Norah J MacMillan, Sophia Kapchinsky, Yana Konokhova, Gilles Gouspillou, Riany de Sousa Sena, R Thomas Jagoe, Jacinthe Baril, Tamara E Carver, Ross E Andersen, Ruddy Richard, Hélène Perrault, Jean Bourbeau, Russell T Hepple, Tanja Taivassalo
Eccentric ergometer training (EET) is increasingly being proposed as a therapeutic strategy to improve skeletal muscle strength in various cardiorespiratory diseases, due to the principle that lengthening muscle actions lead to high force-generating capacity at low cardiopulmonary load. One clinical population that may particularly benefit from this strategy is chronic obstructive pulmonary disease (COPD), as ventilatory constraints and locomotor muscle dysfunction often limit efficacy of conventional exercise rehabilitation in patients with severe disease...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28315692/role-of-epithelial-mesenchymal-transition-emt-and-fibroblast-function-in-cerium-oxide-nanoparticles-induced-lung-fibrosis
#19
Jane Ma, Bridget Bishoff, R R Mercer, Mark Barger, Diane Schwegler-Berry, Vincent Castranova
The emission of cerium oxide nanoparticles (CeO2) from diesel engines, using cerium compounds as a catalyst to lower the diesel exhaust particles, is a health concern. We have previously shown that CeO2 induced pulmonary inflammation and lung fibrosis. The objective of the present study was to investigate the modification of fibroblast function and the role of epithelial-mesenchymal transition (EMT) in CeO2-induced fibrosis. Male Sprague-Dawley rats were exposed to CeO2 (0.15 to 7mg/kg) by a single intratracheal instillation and sacrificed at various times post-exposure...
March 15, 2017: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/28315675/low-intensity-training-and-the-c5a-complement-antagonist-nox-d21-rescue-the-mdx-phenotype-through-modulation-of-inflammation
#20
Janek Hyzewicz, Jun Tanihata, Mutsuki Kuraoka, Yuko Nitahara-Kasahara, Teiva Beylier, Urs T Ruegg, Axel Vater, Shin'ichi Takeda
Inflammatory events occurring in dystrophic muscles contribute to the progression of Duchenne muscular dystrophy (DMD). Low-intensity training (LIT) attenuates the phenotype of mdx mice, an animal model for DMD. Therefore, we postulated that LIT could have anti-inflammatory properties. We assessed levels of inflammatory cytokines and infiltrated immune cells in gastrocnemius muscle of mdx mice after LIT. We detected high levels of complement component C5a, chemokine ligand (CCL) 2, CD68(+) monocytes/macrophages, and proinflammatory M1 macrophages in muscles of mdx mice...
March 15, 2017: American Journal of Pathology
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