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https://www.readbyqxmd.com/read/28488683/progressive-hereditary-spastic-paraplegia-caused-by-a-homozygous-ky-mutation
#1
Yuval Yogev, Yonatan Perez, Iris Noyman, Anwar Abu Madegem, Hagit Flusser, Zamir Shorer, Eugene Cohen, Leonid Kachko, Analia Michaelovsky, Ruth Birk, Arie Koifman, Max Drabkin, Ohad Wormser, Daniel Halperin, Rotem Kadir, Ohad S Birk
Twelve individuals of consanguineous Bedouin kindred presented with autosomal recessive progressive spastic paraplegia evident as of age 0-24 months, with spasticity of lower limbs, hyperreflexia, toe walking and equinus deformity. Kyphoscolisois was evident in older patients. Most had atrophy of the lateral aspects of the tongue and few had intellectual disability. Nerve conduction velocity, electromyography and head and spinal cord magnetic resonance imaging were normal in tested subjects. Muscle biopsy showed occasional central nuclei and fiber size variability with small angular fibers...
May 10, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28470526/quantitative-ratiometric-ca-2-imaging-to-assess-cell-viability
#2
Oliver Friedrich, Stewart I Head
Viability of cells is strongly related to their Ca(2+) homeostasis. Ca(2+) signal fluctuations can be on a slow time scale, e.g., in non-excitable cells, but also in the range of tens of milliseconds for excitable cells, such as nerve and muscle. Muscle fibers respond to electrical stimulation with Ca(2+) transients that exceed their resting basal level about 100 times. Fluorescent Ca(2+) dyes have become an indispensable means to monitor Ca(2+) fluctuations in living cells online. Fluorescence intensity of such "environmental dyes" relies on a buffer-ligand interaction which is not only governed by laws of mass action but also by binding and unbinding kinetics that have to be considered for proper Ca(2+) kinetics and amplitude validation...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28453295/postinjury-exercise-and-platelet-rich-plasma-therapies-improve-skeletal-muscle-healing-in-rats-but-are-not-synergistic-when-combined
#3
Paola Contreras-Muñoz, Joan Ramon Torrella, Xavier Serres, David Rizo-Roca, Meritxell De la Varga, Ginés Viscor, Vicente Martínez-Ibáñez, José Luis Peiró, Tero A H Järvinen, Gil Rodas, Mario Marotta
BACKGROUND: Skeletal muscle injuries are the most common sports-related injury and a major concern in sports medicine. The effect of platelet-rich plasma (PRP) injections on muscle healing is still poorly understood, and current data are inconclusive. PURPOSE: To evaluate the effects of an ultrasound-guided intramuscular PRP injection, administered 24 hours after injury, and/or posttraumatic daily exercise training for 2 weeks on skeletal muscle healing in a recently established rat model of skeletal muscle injury that highly mimics the muscle trauma seen in human athletes...
April 1, 2017: American Journal of Sports Medicine
https://www.readbyqxmd.com/read/28448058/a-rapid-automated-protocol-for-muscle-fiber-population-analysis-in-rat-muscle-cross-sections-using-myosin-heavy-chain-immunohistochemistry
#4
Konstantin D Bergmeister, Marion Gröger, Martin Aman, Anna Willensdorfer, Krisztina Manzano-Szalai, Stefan Salminger, Oskar C Aszmann
Quantification of muscle fiber populations provides a deeper insight into the effects of disease, trauma, and various other influences on skeletal muscle composition. Various time-consuming methods have traditionally been used to study fiber populations in many fields of research. However, recently developed immunohistochemical methods based on myosin heavy chain protein expression provide a quick alternative to identify multiple fiber types in a single section. Here, we present a rapid, reliable and reproducible protocol for improved staining quality, allowing automatic acquisition of whole cross sections and automatic quantification of fiber populations with ImageJ...
March 28, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28431604/a-reappraisal-of-diagnostic-tests-for-myasthenia-gravis-in-a-large-asian-cohort
#5
Yew Long Lo, Raymond P Najjar, Kelvin Y Teo, Sharon L Tow, Jing Liang Loo, Dan Milea
BACKGROUND: Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disease characterized by weakness of bodily skeletal muscles. Office-based diagnostic tests such as repetitive nerve stimulation (RNS), single fiber electromyography (SFEMG), and the ice test, are used to refine the differential clinical diagnosis of this disease. Evaluating the clinical sensitivity and specificity of these tests, however, may be confounded by lack of a gold standard, non-blinding, incorporation bias, use of non-representative populations and retrospective data...
May 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28428630/hinge-deleted-igg4-blocker-therapy-for-acetylcholine-receptor-myasthenia-gravis-in-rhesus-monkeys
#6
Mario Losen, Aran F Labrijn, Vivianne H van Kranen-Mastenbroek, Maarten L Janmaat, Krista G Haanstra, Frank J Beurskens, Tom Vink, Margreet Jonker, Bert A 't Hart, Marina Mané-Damas, Peter C Molenaar, Pilar Martinez-Martinez, Eline van der Esch, Janine Schuurman, Marc H de Baets, Paul W H I Parren
Autoantibodies against ion channels are the cause of numerous neurologic autoimmune disorders. Frequently, such pathogenic autoantibodies have a restricted epitope-specificity. In such cases, competing antibody formats devoid of pathogenic effector functions (blocker antibodies) have the potential to treat disease by displacing autoantibodies from their target. Here, we have used a model of the neuromuscular autoimmune disease myasthenia gravis in rhesus monkeys (Macaca mulatta) to test the therapeutic potential of a new blocker antibody: MG was induced by passive transfer of pathogenic acetylcholine receptor-specific monoclonal antibody IgG1-637...
April 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28397454/histological-evaluation-of-the-local-soft-tissue-reaction-after-implanting-resorbable-and-non-resorbable-monofilament-fibers
#7
Bogusława Żywicka, Maria Szymonowicz, Danuta Bryła, Zbigniew Rybak
BACKGROUND: The development of technologies and scientific disciplines connected with medical implantation devices is dynamically affecting modern treatments by contemporary medicine and veterinary medicine; it also entails a need to monitor their impact on living organisms. OBJECTIVES: The aim of the study was to conduct a comparative histological evaluation of the response of soft tissues after implanting monofilament fibers from resorbable glyconate and from non-resorbable polypropylene (PP) and polyamide (PA) in rats...
July 2016: Polimery W Medycynie
https://www.readbyqxmd.com/read/28384286/t-t-homozygosity-of-the-tenascin-c-gene-polymorphism-rs2104772-negatively-influences-exercise-induced-angiogenesis
#8
Paola Valdivieso, Marco Toigo, Hans Hoppeler, Martin Flück
BACKGROUND: Mechanical stress, including blood pressure related factors, up-regulate expression of the pro-angiogenic extracellular matrix protein tenascin-C in skeletal muscle. We hypothesized that increased capillarization of skeletal muscle with the repeated augmentation in perfusion during endurance training is associated with blood vessel-related expression of tenascin-C and would be affected by the single-nucleotide polymorphism (SNP) rs2104772, which characterizes the non-synonymous exchange of thymidine (T)-to-adenosine (A) in the amino acid codon 1677 of tenascin-C...
2017: PloS One
https://www.readbyqxmd.com/read/28384102/anatomic-variation-of-the-median-nerve-associated-with-an-anomalous-muscle-of-the-forearm
#9
Atoni Dogood Atoni, Charles Aidemise Oyinbo
Documented anatomical variations are important not only for the study of the subject of anatomy, but also in clinical situation. This knowledge would aid surgeons in planning a preoperative strategy for surgical procedures and reconstructive surgery. The right forearm of a 35-year-old embalmed male cadaver present a splitting of the median nerve in the proximal 1/3 of the forearm to form medial and lateral divisions that accommodate an anomalous muscle. The split median nerve reunites at the distal 1/3 and continues as a single nerve...
March 1, 2017: Folia Medica
https://www.readbyqxmd.com/read/28373411/intense-interval-training-in-healthy-older-adults-increases-skeletal-muscle-3-h-ouabain-binding-site-content-and-elevates-na-k-atpase-%C3%AE-2-isoform-abundance-in-type-ii-fibers
#10
Victoria L Wyckelsma, Itamar Levinger, Robyn M Murphy, Aaron C Petersen, Ben D Perry, Christopher P Hedges, Mitchell J Anderson, Michael J McKenna
Young adults typically adapt to intense exercise training with an increased skeletal muscle Na(+),K(+)-ATPase (NKA) content, concomitant with reduced extracellular potassium concentration [K(+)] during exercise and enhanced exercise performance. Whether these changes with longitudinal training occur in older adults is unknown and was investigated here. Fifteen older adults (69.4 ± 3.5 years, mean ± SD) were randomized to either 12 weeks of intense interval training (4 × 4 min at 90-95% peak heart rate), 3 days/week (IIT, n = 8); or no exercise controls (n = 7)...
April 2017: Physiological Reports
https://www.readbyqxmd.com/read/28370195/axonal-domain-disorganization-in-caspr1-and-caspr2-mutant-myelinated-axons-affects-neuromuscular-junction-integrity-leading-to-muscle-atrophy
#11
Julia Saifetiarova, Xi Liu, Anna M Taylor, Jie Li, Manzoor A Bhat
Bidirectional interactions between neurons and myelinating glial cells result in formation of axonal domains along myelinated fibers. Loss of axonal domains leads to detrimental consequences on nerve structure and function, resulting in reduced conductive properties and the diminished ability to reliably transmit signals to the targets they innervate. Thus, impairment of peripheral myelinated axons that project to the surface of muscle fibers and form neuromuscular junction (NMJ) synapses leads to muscle dysfunction...
July 2017: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/28360784/jitter-values-on-voluntary-active-periocular-muscles-of-healthy-subjects-with-conventional-37-mm-concentric-needle-electrode
#12
Leyla Baysal Kiraç, Elif Kocasoy Orhan, Saygın Gönderten, Mehmet Barış Baslo, Ali Emre Öge
INTRODUCTION: The aim of this study was to re-evaluate jitter values of healthy subjects in whom pairs of single-fiber-like potentials were recorded from voluntary activated periocular muscles using a disposable 37-mm concentric needle electrode (CNE) with 2-kHz low-cut filtering. METHODS: We reviewed the recordings of 129 subjects (85 women; 44 men; mean age, 43.8±15.3 years). The m. frontalis group included 116 subjects, and the m. orbicularis oculi group included 18 subjects...
June 2016: Noro Psikiyatri Arsivi
https://www.readbyqxmd.com/read/28350570/low-density-lipoprotein-receptor-related-protein-4-positive-myasthenia-gravis-in-a-double-seronegative-electromyography-negative-patient
#13
Joshua M Kruger, Dimitrios Karussis, Paraskevi Zisimopoulou, Panayiota Petrou
We describe a patient with ocular myasthenia gravis, where single-fiber electromyography and testing for acetylcholine receptor and muscle-specific kinase antibodies were negative. However, antibodies to low-density lipoprotein receptor-related protein 4 (LRP4) were positive, and this prompted appropriate management. We recommend that testing for LRP4 antibodies be considered when the clinical suspicion for myasthenia gravis is high despite negative conventional diagnostic tests.
March 27, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/28345221/effect-of-timing-of-electromyostimulation-on-denervated-skeletal-muscle-atrophy
#14
Eun Sil Koh, Hee Chan Kim, Jae-Young Lim
INTRODUCTION: In this study we evaluated the effect of electromyostimulation (EMS) on myosin heavy chain (MHC) isoform expression in denervated rat muscles to determine the optimal timing for EMS application. METHODS: EMS was initiated on post-injury day 1 for the group with denervation receiving immediate EMS (DIEMS) and on post-injury day 15 for the group with denervation receiving delayed EMS (DDEMS) in rat denervated muscles. Muscle wet weight and muscle fiber cross-sectional area (FCSA) were measured...
March 27, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28315692/role-of-epithelial-mesenchymal-transition-emt-and-fibroblast-function-in-cerium-oxide-nanoparticles-induced-lung-fibrosis
#15
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...
May 15, 2017: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/28298356/the-glass-micropipette-electrode-a-history-of-its-inventors-and-users-to-1950
#16
Allan H Bretag
Soon after the glass micropipette was invented as a micro-tool for manipulation of single bacteria and the microinjection and microsurgery of living cells, it was seen to hold promise as a microelectrode to stimulate individual cells electrically and to study electrical potentials in them. Initial successes and accurate mechanistic explanations of the results were achieved in giant plant cells in the 1920s. Long known surface electrical activity in nerves and muscles was only resolved at a similar cellular level in the 1930s and 1940s after the discovery of giant nerve fibers and the development of finer tipped microelectrodes for normal-sized cells...
April 3, 2017: Journal of General Physiology
https://www.readbyqxmd.com/read/28292875/in-vivo-ca-2-dynamics-induced-by-ca-2-injection-in-individual-rat-skeletal-muscle-fibers
#17
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/28281032/mouse-soleus-slow-muscle-shows-greater-intramyocellular-lipid-droplet-accumulation-than-edl-fast-muscle-fiber-type-specific-analysis
#18
Yusuke Komiya, Shoko Sawano, Daisuke Mashima, Riho Ichitsubo, Mako Nakamura, Ryuichi Tatsumi, Yoshihide Ikeuchi, Wataru Mizunoya
Skeletal muscle is the main tissue of lipid metabolism and accordingly is critical for homeostasis and energy production; however, the determinants of lipid accumulation in skeletal muscle are unknown. Here, we examined whether the soleus muscle (predominantly slow-twitch fibers) has a higher lipid accumulation capacity than that of the extensor digitorum longus (EDL, predominantly fast-twitch fibers) muscle in mice. Soleus and EDL muscles were harvested from male C57BL/6J mice. The mRNA levels of genes involved in fatty acid import and triglyceride synthesis and accumulation were examined in soleus and EDL muscles...
March 9, 2017: Journal of Muscle Research and Cell Motility
https://www.readbyqxmd.com/read/28270752/neuronal-correlates-of-functional-coupling-between-reach-and-grasp-related-components-of-muscle-activity
#19
Shashwati Geed, Martha L McCurdy, Peter L E van Kan
Coordinated reach-to-grasp movements require precise spatiotemporal synchrony between proximal forelimb muscles (shoulder, elbow) that transport the hand toward a target during reach, and distal muscles (wrist, digit) that simultaneously preshape and orient the hand for grasp. The precise mechanisms through which the redundant neuromuscular circuitry coordinates reach with grasp, however, remain unclear. Recently, Geed and Van Kan (2016) demonstrated, using exploratory factor analysis (EFA), that limited numbers of global, template-like transport/preshape- and grasp-related muscle components underlie the complexity and variability of intramuscular electromyograms (EMGs) of up to 21 distal and proximal muscles recorded while monkeys performed reach-to-grasp tasks...
2017: Frontiers in Neural Circuits
https://www.readbyqxmd.com/read/28268468/detection-of-myasthenia-gravis-using-electrooculography-signals
#20
T Liang, M I Boulos, B J Murray, S Krishnan, H Katzberg, K Umapathy
Myasthenia gravis (MG) is an autoimmune neuromuscular disorder resulting from skeletal muscle weakness and fatigue. An early common symptom is fatigable weakness of the extrinsic ocular muscles; if symptoms remain confined to the ocular muscles after a few years, this is classified as ocular myasthenia gravis (OMG). Diagnosis of MG when there are mild, isolated ocular symptoms can be difficult, and currently available diagnostic techniques are insensitive, non-specific or technically cumbersome. In addition, there are no accurate biomarkers to follow severity of ocular dysfunction in MG over time...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
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