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https://www.readbyqxmd.com/read/28937031/a-novel-agrn-mutation-leads-to-congenital-myasthenic-syndrome-only-affecting-limb-girdle-muscle
#1
Ying Zhang, Yi Dai, Jing-Na Han, Zhao-Hui Chen, Li Ling, Chuan-Qiang Pu, Li-Ying Cui, Xu-Sheng Huang
BACKGROUND: Congenital myasthenic syndromes (CMSs) are a group of clinically and genetically heterogeneous disorders caused by impaired neuromuscular transmission. The defect of AGRN was one of the causes of CMS through influencing the development and maintenance of neuromuscular transmission. However, CMS reports about this gene mutation were rare. Here, we report a novel homozygous missense mutation (c.5302G>C) of AGRN in a Chinese CMS pedigree. METHODS: We performed a detailed clinical assessment of a Chinese family with three affected members...
October 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28934327/a-novel-miniature-in-line-load-cell-to-measure-in-situ-tensile-forces-in-the-tibialis-anterior-tendon-of-rats
#2
Martin Schmoll, Ewald Unger, Manfred Bijak, Martin Stoiber, Hermann Lanmüller, Jonathan Charles Jarvis
Direct measurements of muscular forces usually require a substantial rearrangement of the biomechanical system. To circumvent this problem, various indirect techniques have been used in the past. We introduce a novel direct method, using a lightweight (~0.5 g) miniature (3 x 3 x 7 mm) in-line load-cell to measure tension in the tibialis anterior tendon of rats. A linear motor was used to produce force-profiles to assess linearity, step-response, hysteresis and frequency behavior under controlled conditions...
2017: PloS One
https://www.readbyqxmd.com/read/28932757/a-five-repeat-micro-dystrophin-gene-ameliorated-dystrophic-phenotype-in-the-severe-dba-2j-mdx-model-of-duchenne-muscular-dystrophy
#3
Chady H Hakim, Nalinda B Wasala, Xiufang Pan, Kasun Kodippili, Yongping Yue, Keqing Zhang, Gang Yao, Brittney Haffner, Sean X Duan, Julian Ramos, Joel S Schneider, N Nora Yang, Jeffrey S Chamberlain, Dongsheng Duan
Micro-dystrophins are highly promising candidates for treating Duchenne muscular dystrophy, a lethal muscle disease caused by dystrophin deficiency. Here, we report robust disease rescue in the severe DBA/2J-mdx model with a neuronal nitric oxide synthase (nNOS)-binding micro-dystrophin vector. 2 × 10(13) vector genome particles/mouse of the vector were delivered intravenously to 10-week-old mice and were evaluated at 6 months of age. Saturated micro-dystrophin expression was detected in all skeletal muscles and the heart and restored the dystrophin-associated glycoprotein complex and nNOS...
September 15, 2017: Molecular Therapy. Methods & Clinical Development
https://www.readbyqxmd.com/read/28930932/complex-training-the-effect-of-exercise-selection-and-training-status-on-postactivation-potentiation-in-rugby-league-players
#4
David J Scott, Massimiliano Ditroilo, Phil A Marshall
Scott, DJ, Ditroilo, M, and Marshall, PA. Complex training: the effect of exercise selection and training status on postactivation potentiation in rugby league players. J Strength Cond Res 31(10): 2694-2703, 2017-This study compared the postactivation potentiation (PAP) response of the hex bar deadlift (HBD) and back squat (BS) exercises. The PAP response between different levels of athletes was also compared. Ten professional and 10 amateur rugby league players performed 2 experimental sessions. Participants performed a countermovement jump (CMJ) before and 2, 4, 6, 8, 10, 12, 14, and 16 minutes after a conditioning activity (CA) that contained 1 set of 3 repetitions at 93% 1 repetition maximum of either HBD or BS...
October 2017: Journal of Strength and Conditioning Research
https://www.readbyqxmd.com/read/28930864/neural-correlates-to-the-increase-in-maximal-force-after-dexamethasone-administration
#5
Stéphane Baudry, Alberto Botter, Jacques Duchateau, Marco A Minetto
PURPOSE: This study investigated the effects of short-term glucocorticoid administration on voluntary activation and intracortical inhibitory and facilitatory circuits. METHODS: Seventeen healthy men participated in a pseudo randomized double-blind study to receive either dexamethasone (8 mg·d, n = 9 subjects) or placebo (n = 8 subjects) for 7 days. The ankle dorsiflexion torque, corresponding electromyography (EMG) of the tibialis anterior, and voluntary activation assessed by the interpolated twitch method using transcranial magnetic stimulation (TMS) were measured during a maximal voluntary contraction (MVC)...
September 19, 2017: Medicine and Science in Sports and Exercise
https://www.readbyqxmd.com/read/28921721/effects-of-the-availability-of-accurate-proprioceptive-information-on-older-adults-postural-sway-and-muscle-co-contraction
#6
Chesney E Craig, Glenn H M Calvert, Michail Doumas
During conditions of increased postural instability, older adults exhibit greater lower-limb muscle co-contraction. This response has been interpreted as a compensatory postural strategy, which may be used to i006Ecrease proprioceptive information from muscle spindles or to stiffen the lower limb as a general response to minimise postural sway. The current study aimed to test these two hypotheses by investigating use of muscle co-contraction during sensory transitions that manipulated proprioceptive input. Surface EMG was recorded from the bilateral tibialis anterior and gastrocnemius medialis muscles, in young (aged 18-30) and older adults (aged 68-80) during blind-folded postural assessment...
September 16, 2017: European Journal of Neuroscience
https://www.readbyqxmd.com/read/28918357/long-term-follow-up-after-tibialis-anterior-tendon-shortening-in-combination-with-achilles-tendon-lengthening-in-spastic-equinus-in-cerebral-palsy
#7
Michèle Kläusler, Bernhard Maria Speth, Reinald Brunner, Oren Tirosh, Carlo Camathias, Erich Rutz
Using Tibialis Anterior Shortening (TATS) in combination with Achilles Tendon Lengthening (TAL) to treat spastic equinus in children with cerebral palsy (CP) was described in 2011. Short-term results have indicated a good outcome, especially an improvement of the drop foot in swing phase and the correction of equinus in stance phase. The aim of this study was to analyse the results of the long-term follow-up and to determine the relapse rate of TATS and TAL. The kinematics of the sagittal, frontal and transversal planes were measured by using instrumented 3D gait analysis at three defined time points and then described using the Gait Profile Score (GPS) and Movement Analysis Profile (MAP)...
August 30, 2017: Gait & Posture
https://www.readbyqxmd.com/read/28917641/changes-in-innervation-of-lumbar-motoneurons-and-organization-of-premotor-network-following-training-of-transected-adult-rats
#8
Loubna Khalki, Karina Sadlaoud, Julie Lerond, Jacques-Olivier Coq, Jean-Michel Brezun, Laurent Vinay, Patrice Coulon, Hélène Bras
Rats with complete spinal cord transection (SCT) can recover hindlimb locomotor function under strategies combining exercise training and 5-HT agonist treatment. This recovery is expected to result from structural and functional re-organization within the spinal cord below the lesion. To begin to understand the nature of this reorganization, we examined synaptic changes to identified gastrocnemius (GS) or tibialis anterior (TA) motoneurons (MNs) in SCT rats after a schedule of early exercise training and delayed 5-HT agonist treatment...
September 13, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28914616/effect-of-full-length-carbon-fiber-insoles-on-lower-limb-kinetics-in-patients-with-midfoot-osteoarthritis-a-pilot-study
#9
Taeim Yi, Jung Hyun Kim, Mooyeon Oh-Park, Ji Hye Hwang
OBJECTIVES: We investigated the effects of full-length carbon fiber (FCF) insoles on gait, muscle activity, kinetics, and pain in patients with midfoot osteoarthritis (OA). DESIGN: We enrolled 13 patients with unilateral midfoot OA (mild: Visual Analog Scale [VAS] range, 1-3; moderate, VAS range, 4-7) and healthy controls. All participants were asked to walk under two conditions: with and without FCF insole. The outcome measures were ground reaction force, quantitative gait parameters, electromyography activities and pain severity (VAS)...
September 14, 2017: American Journal of Physical Medicine & Rehabilitation
https://www.readbyqxmd.com/read/28912629/spinal-manipulation-does-not-affect-pressure-pain-thresholds-in-the-absence-of-neuromodulators-a-randomized-controlled-trial
#10
Max K Jordon, Paul F Beattie, Sarah D'Urso, Sarah Scriven
BACKGROUND: Measurement of pressure pain threshold (PPT) is a way to determine one of the many potential treatment effects of spinal manipulative therapy. OBJECTIVE: To determine how multiple spinal manipulations administered in a single-session affected PPTs at local and distal sites in asymptomatic individuals. METHODS: Participants were randomly assigned into one of three groups: Group one (n = 18) received a lumbar manipulation followed by a cervical manipulation...
September 2017: Journal of Manual & Manipulative Therapy
https://www.readbyqxmd.com/read/28910370/intra-and-inter-session-reliability-of-rapid-transcranial-magnetic-stimulation-stimulus-response-curves-of-tibialis-anterior-muscle-in-healthy-older-adults
#11
Elisabetta Peri, Emilia Ambrosini, Vera Maria Colombo, Mark van de Ruit, Michael J Grey, Marco Monticone, Giorgio Ferriero, Alessandra Pedrocchi, Giancarlo Ferrigno, Simona Ferrante
OBJECTIVE: The clinical use of Transcranial Magnetic Stimulation (TMS) as a technique to assess corticospinal excitability is limited by the time for data acquisition and the measurement variability. This study aimed at evaluating the reliability of Stimulus-Response (SR) curves acquired with a recently proposed rapid protocol on tibialis anterior muscle of healthy older adults. METHODS: Twenty-four neurologically-intact adults (age:55-75 years) were recruited for this test-retest study...
2017: PloS One
https://www.readbyqxmd.com/read/28904095/distinct-roles-for-motor-neuron-autophagy-early-and-late-in-the-sod1-g93a-mouse-model-of-als
#12
Noam D Rudnick, Christopher J Griffey, Paolo Guarnieri, Valeria Gerbino, Xueyong Wang, Jason A Piersaint, Juan Carlos Tapia, Mark M Rich, Tom Maniatis
Mutations in autophagy genes can cause familial and sporadic amyotrophic lateral sclerosis (ALS). However, the role of autophagy in ALS pathogenesis is poorly understood, in part due to the lack of cell type-specific manipulations of this pathway in animal models. Using a mouse model of ALS expressing mutant superoxide dismutase 1 (SOD1(G93A)), we show that motor neurons form large autophagosomes containing ubiquitinated aggregates early in disease progression. To investigate whether this response is protective or detrimental, we generated mice in which the critical autophagy gene Atg7 was specifically disrupted in motor neurons (Atg7 cKO)...
September 13, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28900414/alterations-in-spectral-attributes-of-surface-electromyograms-after-utilization-of-a-foot-drop-stimulator-during-post-stroke-gait
#13
Rakesh Pilkar, Arvind Ramanujam, Karen J Nolan
BACKGROUND: A foot drop stimulator (FDS) is a rehabilitation intervention that stimulates the common peroneal nerve to facilitate ankle dorsiflexion at the appropriate time during post-stroke hemiplegic gait. Time-frequency analysis (TFA) of non-stationary surface electromyograms (EMG) and spectral variables such as instantaneous mean frequency (IMNF) can provide valuable information on the long-term effects of FDS intervention in terms of changes in the motor unit (MU) recruitment during gait, secondary to improved dorsiflexion...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28895461/skeletal-muscle-atrophy-and-degeneration-in-a-mouse-model-of-traumatic-brain-injury
#14
Bahar Shahidi, Sameer B Shah, Mary Esparza, Brian Patrick Head, Sam Ward
Atrophy is thought to be a primary mode of muscle loss in neuromuscular injuries. The differential effects of central and peripheral injuries on atrophy and degeneration/regeneration in skeletal muscle tissue have not been well described. This study investigated skeletal muscle atrophy and degeneration/regeneration in an animal model of traumatic brain injury (TBI). Eight 8-month old wild type C57BL6 mice underwent either a sham craniotomy or TBI targeting the motor cortex. Atrophy (Fiber Area; FA) and degeneration/regeneration (Centralized Nuclei proportions; CN) of the Soleus and Tibialis Anterior (TA) muscles were measured 2 months post injury...
September 12, 2017: Journal of Neurotrauma
https://www.readbyqxmd.com/read/28892032/semi-automated-analysis-of-mouse-skeletal-muscle-morphology-and-fiber-type-composition
#15
Sidharth Tyagi, Donald Beqollari, Chang Seok Lee, Lori A Walker, Roger A Bannister
For years, distinctions between skeletal muscle fiber types were best visualized by myosin-ATPase staining. More recently, immunohistochemical staining of myosin heavy chain (MyHC) isoforms has emerged as a finer discriminator of fiber-type. Type I, type IIA, type IIX and type IIB fibers can now be identified with precision based on their MyHC profile; however, manual analysis of these data can be slow and down-right tedious. In this regard, rapid, accurate assessment of fiber-type composition and morphology is a very desirable tool...
August 31, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28888906/the-comparison-of-ground-reaction-forces-and-lower-limb-muscles-correlation-and-activation-time-delay-between-forward-and-backward-walking
#16
Mohammadreza Mahaki, Gustavo Souto De Sá E Souza, Raghad Mimar, Marcus Fraga Vieira
This study aimed to compare the ground reaction forces (GRF) and lower limb muscles correlation and activation time delay between Forward (FW) and Backward (BW) walking. Twenty-four male students participated in this research. Electromyogram activities of gluteus medius, biceps femoris, medial gastrocnemius, soleus and anterior tibialis muscles along with GRFs were measured. Each participant performed two FW and two BW trials bare foot. Statistical parametric mapping (SPM) analysis was performed over anterior-posterior and vertical GRFs time series...
September 6, 2017: Gait & Posture
https://www.readbyqxmd.com/read/28887003/effects-of-acupuncture-at-the-st-36-point-on-muscle-sympathetic-nerve-activity-and-blood-pressure-in-normal-adults
#17
Kenichi Kimura, Kazuya Ishida, Noriyo Takahashi, Yasushi Toge, Fumihiro Tajima
The aim of the present study was to determine the effects of acupuncture on post-ganglionic muscle sympathetic nerve activity (MSNA) in humans. MSNA was measured in 8 healthy adult males by microneurography evaluation of the left peroneal nerve. Blood pressure (BP) and heart rate (HR) were simultaneously recorded. MSNA was evaluated as the burst rate, with total MSNA, BP and HR normalized to their respective baseline values. After 10min of rest in the supine position, acupuncture was applied to the right ST-36 point in the tibialis anterior muscle for 15min, with recovery then monitored over a 20-min period...
September 1, 2017: Autonomic Neuroscience: Basic & Clinical
https://www.readbyqxmd.com/read/28886464/muscle-activity-during-backward-and-forward-running-with-body-weight-support
#18
Kenji Masumoto, Michael T Soucy, Joshua P Bailey, John A Mercer
We investigated muscle activity during backward (BR) and forward (FR) running with body weight support (BWS). Ten participants completed BR and FR on a lower body positive pressure treadmill while selecting a preferred speed (PS) for different BWS conditions (0%, 20%, 40%, 60%, and 80%BWS). Muscle activity from the rectus femoris (RF), biceps femoris (BF), tibialis anterior (TA), and gastrocnemius (GA), rating of perceived exertion (RPE), preferred stride frequency (PSF), and PS were measured. Magnitude of muscle activity (BF, TA, and GA), RPE, PSF, and PS were not influenced by the interaction of direction and BWS (P>0...
September 5, 2017: Human Movement Science
https://www.readbyqxmd.com/read/28872527/muscle-microvascular-blood-flow-oxygenation-ph-and-perfusion-pressure-decrease-in-simulated-acute-compartment-syndrome
#19
Sravya T Challa, Alan R Hargens, Amarachi Uzosike, Brandon R Macias
BACKGROUND: The current gold standard for diagnosing acute compartment syndrome (ACS) is an assessment of clinical signs, invasive measurement of intramuscular pressure (IMP), and measurement of local perfusion pressure. However, IMP measurements have several shortcomings, including pain, risk of infection, risk of technique error, plugging of the catheter tip, lack of consensus on the diagnostic pressure threshold, and lack of specificity and sensitivity. The objective of this study was to evaluate muscle hemodynamics, oxygenation, and pH as diagnostic parameters in a human model of ACS...
September 6, 2017: Journal of Bone and Joint Surgery. American Volume
https://www.readbyqxmd.com/read/28872121/minimally-invasive-muscle-embedding-mime-a-novel-experimental-technique-to-facilitate-donor-cell-mediated-myogenesis
#20
Joseph A Roche, Morium Begam, Sujay S Galen
Skeletal muscle possesses regenerative capacity due to tissue-resident, muscle-fiber-generating (myogenic) satellite cells (SCs), which can form new muscle fibers under the right conditions. Although SCs can be harvested from muscle tissue and cultured in vitro, the resulting myoblast cells are not very effective in promoting myogenesis when transplanted into host muscle. Surgically exposing the host muscle and grafting segments of donor muscle tissue, or the isolated muscle fibers with their SCs onto host muscle, promotes better myogenesis compared to myoblast transplantation...
August 24, 2017: Journal of Visualized Experiments: JoVE
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