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https://www.readbyqxmd.com/read/29353265/growth-sexual-and-bone-development-in-a-boy-with-bilateral-anorchia-under-testosterone-treatment-guided-by-the-development-of-his-monozygotic-twin
#1
Sara Vandewalle, Eva Van Caenegem, Margarita Craen, Youri Taes, Jean-Marc Kaufman, Guy T'Sjoen
BACKGROUND: Sex steroids are essential for sexual maturation, linear growth and bone development. However, there is no consensus on the optimal timing, dosage and dosage interval of testosterone therapy to induce pubertal development and achieve a normal adult height and bone mass in children with hypogonadism. CASE PRESENTATION: A monozygotic monochorial male twin pair, of which one boy was diagnosed with anorchia at birth due to testicular regression syndrome was followed from the age of 3 until the age of 18 years...
January 20, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29353241/inhibition-of-endothelial-notch-signaling-impairs-fatty-acid-transport-and-leads-to-metabolic-and-vascular-remodeling-of-the-adult-heart
#2
Markus Jabs, Adam J Rose, Lorenz H Lehmann, Jacqueline Taylor, Iris Moll, Tjeerd P Sijmonsma, Stefanie E Herberich, Sven W Sauer, Gernot Poschet, Giuseppina Federico, Carolin Mogler, Eva-Maria Weis, Hellmut G Augustin, Minhong Yan, Norbert Gretz, Roland M Schmid, Ralf H Adams, Hermann-Joseph Gröne, Rüdiger Hell, Jürgen G Okun, Johannes Backs, Peter P Nawroth, Stephan Herzig, Andreas Fischer
Background -Nutrients are transported through endothelial cells before being metabolized in muscle cells. However, little is known about the regulation of endothelial transport processes. Notch signaling is a critical regulator of metabolism and angiogenesis during development. Here, we studied how genetic and pharmacological manipulation of endothelial Notch signaling in adult mice affects endothelial fatty acid transport, cardiac angiogenesis, and heart function. Methods -Endothelial-specific Notch inhibition was achieved by conditional genetic inactivation of Rbp-jκ in adult mice to analyze fatty acid metabolism and heart function...
January 20, 2018: Circulation
https://www.readbyqxmd.com/read/29353205/wisp3-prevents-fibroblast-myofibroblast-transdifferentiation-in-nrk-49f-cells
#3
Yang Yi, Jun Ma, Lu Jianrao, Hangqing Wang, Yingdan Zhao
CCN family, a group of six extracellular matrix-associated proteins, plays an important role in fibrosis. WISP3 has addressed as a pro-fibrotic molecule in the development of human lung fibrosis. However, whether WISP3 involved in the activation and proliferation of renal fibroblast, and ultimately inhibited fibroblast-myofibroblast transdifferentiation remained unknown. Herein, we found that down-regulated WISP3 was involved in the fibrogenesis of rat renal NRK-49F cells induced by transforming growth (TGF-β1), which was further confirmed in a rat renal fibrosis induced by unilateral ureteral obstruction (UUO)...
January 17, 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29353180/differential-changes-in-the-spinal-segmental-locomotor-output-in-hereditary-spastic-paraplegia
#4
G Martino, Y Ivanenko, M Serrao, A Ranavolo, F Draicchio, M Rinaldi, C Casali, F Lacquaniti
OBJECTIVE: A comprehensive treatment of Hereditary Spastic Paraplegia (HSP) should consider the specific pathophysiological changes in the spinal cord. Here we reported a detailed characterization of the spinal motoneuronal output in HSP during locomotion. METHODS: We recorded kinematics and electromyographic (EMG) activity of 12 leg muscles in 29 patients with pure forms of HSP and compared them with 30 controls while walking at matched speeds. We assessed the spinal locomotor output by evaluating EMG patterns and by mapping them onto the rostrocaudal location of the spinal motoneuron pools...
December 23, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29353169/integrated-biomarkers-response-confirm-the-antioxidant-role-of-diphenyl-diselenide-against-atrazine
#5
Aline Teixeira Marins, Cíntia Côrte Real Rodrigues, Charlene Cavalheiro de Menezes, Jeane de Lima Costa Gomes, Maiara Dorneles Costa, Mauro Eugênio Medina Nunes, Mariela de Souza Vieira, Filipe Fagan Donato, Renato Zanella, Leila Picolli da Silva, Vania Lucia Loro
Atrazine (ATZ) is a herbicide worldwide used. That can cause oxidative damage in non-target organisms, such as fish. Furthermore, the threat of exposure to pesticides together with poor nutrition is hazardous to the normal development of fish, and supplementation of the fish diet with antioxidants compounds is an alternative approach to prevent the hazardous effects of pesticide exposure. Here we aimed to investigate the capacity of diphenyl diselenide (PhSe)2 diet supplementation to improve the antioxidant defense of Cyprinus carpio (carp) exposed to environmental concentrations of ATZ...
January 16, 2018: Ecotoxicology and Environmental Safety
https://www.readbyqxmd.com/read/29353138/competitive-swimmers-with-hypermobility-have-strength-and-fatigue-deficits-in-shoulder-medial-rotation
#6
Behnam Liaghat, Birgit Juul-Kristensen, Thomas Frydendal, Camilla Marie Larsen, Karen Søgaard, Aki Ilkka Tapio Salo
Generalised Joint Hypermobility including shoulder hypermobility (GJHS) in swimmers is considered an intrinsic risk factor for shoulder injuries. The aim was to investigate the association of GJHS with shoulder strength, fatigue development and muscle activity during swimming-related shoulder rotations. Totally, 38 competitive swimmers (aged 13-17 years) participated, 19 were competitive swimmers with GJHS and 19 were age, sex and club matched swimmers without GJHS. Concentric isokinetic force in medial and lateral rotations were measured at 60°/s (5 repetitions) and 180°/s (10 repetitions)...
January 10, 2018: Journal of Electromyography and Kinesiology
https://www.readbyqxmd.com/read/29352975/pinocembrin-from-penthorum-chinense-pursh-suppresses-hepatic-stellate-cells-activation-through-a-unified-sirt3-tgf-%C3%AE-smad-signaling-pathway
#7
Fayang Zhou, Anqi Wang, Dan Li, Yitao Wang, Ligen Lin
The inactivation of hepatic stellate cells (HSCs) has been verified to be an effective therapeutic strategy for treatment of liver fibrosis. Penthorum chinense Pursh has been widely used to protect liver in China; while, the role of P. chinense Pursh in treatment of liver fibrosis is still unexplored. In the current study, the aqueous extract of P. chinense Pursh (PCE) was found to suppress the expressions of fibrotic markers, including collagen I and α-smooth muscle actin (α-SMA), in human HSCs (LX-2); and its major active constituent, pinocembrin (PIN), was discovered to inhibit the expressions of fibrotic markers in LX-2 cells and rat HSCs (HSC-T6)...
January 15, 2018: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/29352858/cardiac-repair-by-epicardial-emt-current-targets-and-a-potential-role-for-the-primary-cilium
#8
REVIEW
Jessica N Blom, Qingping Feng
Despite therapeutic advances that have prolonged life, myocardial infarction (MI) remains a leading cause of death worldwide and imparts a significant economic burden. The advancement of treatments to improve cardiac repair post-MI requires the discovery of new targeted treatment strategies. Recent studies have highlighted the importance of the epicardial covering of the heart in both cardiac development and lower vertebrate cardiac regeneration. The epicardium serves as a source of cardiac cells including smooth muscle cells, endothelial cells and cardiac fibroblasts...
January 17, 2018: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29352809/a-novel-plec-nonsense-homozygous-mutation-c-7159g%C3%A2-%C3%A2-t-p-glu2387-causes-epidermolysis-bullosa-simplex-with-muscular-dystrophy-and-diffuse-alopecia-a-case-report
#9
Zoe Argyropoulou, Lu Liu, Linda Ozoemena, Claudia C Branco, Raquel Senra, Ângela Reis-Rego, Luisa Mota-Vieira
BACKGROUND: Epidermolysis bullosa simplex with muscular dystrophy (EBS-MD; OMIM #226670) is an autosomal recessive disease, characterized mainly by skin blistering at birth or shortly thereafter, progressive muscle weakness, and rarely by alopecia. EBS-MD is caused by mutations in the PLEC gene (OMIM *601282), which encodes plectin, a structural protein expressed in several tissues, including epithelia and muscle. We describe a patient affected with EBS-MD and diffuse alopecia in which we identified a novel pathogenic mutation by PCR amplification of all coding exons and exon-intron boundaries of PLEC gene, followed by bidirectional Sanger sequencing...
January 20, 2018: BMC Dermatology
https://www.readbyqxmd.com/read/29351619/repairing-folding-defective-%C3%AE-sarcoglycan-mutants-by-cftr-correctors-a-potential-therapy-for-limb-girdle-muscular-dystrophy-2d
#10
Marcello Carotti, Justine Marsolier, Michela Soardi, Elisa Bianchini, Chiara Gomiero, Chiara Fecchio, Sara F Henriques, Romeo Betto, Roberta Sacchetto, Isabelle Richard, Dorianna Sandonà
Limb Girdle Muscular Dystrophy type 2D (LGMD2D) is a rare autosomal-recessive disease, affecting striated muscle, due to mutation of SGCA, the gene coding for α-sarcoglycan. Nowadays more than 50 different SGCA missense mutations have been reported. They are supposed to impact folding and trafficking of α-sarcoglycan because the defective polypeptide, although potentially functional, is recognized and disposed of by the quality control of the cell. The secondary reduction of α-sarcoglycan partners, β-, γ- and δ-sarcoglycan, disrupts a key membrane complex that, associated to dystrophin, contributes to assure sarcolemma stability during muscle contraction...
January 16, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29351598/smooth-muscle-cells-of-intracranial-vessels-from-development-to-disease
#11
Juhana Frösen, Anne Joutel
Cerebrovascular diseases that cause ischemic or hemorrhagic stroke with subsequent loss of life or functional capacity due to damage of the brain tissue are among the leading causes of human suffering and economic burden inflicted by diseases in the developed world. Diseases affecting intracranial vessels are significant contributors to ischemic and hemorrhagic strokes. Brain arteriovenous malformations (bAVM), which are a collection of abnormal blood vessels connecting arteries to veins, are the most common cause of intracranial hemorrhage in children and young adults...
January 16, 2018: Cardiovascular Research
https://www.readbyqxmd.com/read/29351462/maximal-strength-training-induced-improvements-in-forearm-work-efficiency-are-associated-with-reduced-blood-flow
#12
Ole Kristian Berg, Stian Kwak Nyberg, Tobias Midtvedt Windedal, Eivind Wang
Maximal strength training (MST) improves work efficiency. However, since blood flow is greatly dictated by muscle contractions in arms during exercise, and vascular conductance is lower, it has been indicated that arms rely more upon adapting oxygen extraction than legs in response to the enhanced work efficiency. Thus, to investigate if metabolic and vascular responses are arm-specific, we utilized Doppler-ultrasound and a catheter placed in the subclavian vein to measure blood flow and a-vO2diff during steady state work in seven young males (24{plus minus}3(SD) years) following six-weeks of handgrip MST...
December 29, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/29351458/differences-in-l-type-calcium-channel-activity-partially-underlie-the-regional-dichotomy-in-pumping-behavior-by-murine-peripheral-and-visceral-lymphatic-vessels
#13
Scott D Zawieja, Jorge A Castorena-Gonzalez, Joshua Scallan, Michael J Davis
We identified a regional dichotomy in murine lymphatic contractile function with regard to vessel location within either the periphery or visceral cavity. All vessels isolated from peripheral regions; cervical, popliteal, inguinal, axillary, and inter-nodal inguinal axillary (Ing-Ax) developed robust contractions with maximal ejection fractions (EFs) of 50-80% in our ex vivo isobaric myograph experiments. Conversely, vessels isolated from the visceral cavity (mesenteric, TD, and iliac) demonstrated maximal EFs below 15%...
January 5, 2018: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/29351457/important-role-of-endothelium-dependent-hyperpolarization-in-pulmonary-microcirculation-in-male-mice-implications-for-hypoxia-induced-pulmonary-hypertension
#14
Shuhei Tanaka, Takashi Shiroto, Shigeo Godo, Hiroki Saito, Yosuke Ikumi, Akiyo Ito, Shoko Kajitani, Saori Sato, Hiroaki Shimokawa
Endothelium-dependent hyperpolarization (EDH) plays important roles in systemic circulation, whereas its role in pulmonary circulation remains largely unknown. Furthermore, the underlying mechanisms of pulmonary hypertension (PH) also remain to be elucidated. We thus aimed to elucidate the role of EDH in pulmonary circulation in general and in PH in particular. In isolated perfused lung using male wild-type mice, endothelium-dependent relaxations to bradykinin (BK) were significantly reduced in the presence of Nω-nitro-L-arginine (L-NNA) by ~50% as compared with those in the presence of indomethacin, and the combination of apamin plus charybdotoxin abolished the residual relaxations, showing the comparable contributions of nitric oxide (NO) and EDH in pulmonary microcirculation under physiological conditions...
January 5, 2018: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/29351412/expression-of-ctgf-ccn2-in-response-to-lpa-is-stimulated-by-fibrotic-extracellular-matrix-via-the-integrin-fak-axis
#15
Camilo Riquelme-Guzman, Osvaldo Contreras, Enrique Brandan
Fibrosis is a common feature of several chronic diseases, and is characterized by exacerbated accumulation of extracellular matrix (ECM). Understanding the cellular and molecular mechanisms involved in the development of this condition is crucial for designing efficient treatments for those pathologies. Connective tissue growth factor (CTGF/CCN2) is a pleiotropic protein with strong pro-fibrotic activity. In this report, we present experimental evidence showing that ECM stimulates the synthesis of CTGF in response to lysophosphatidic acid (LPA)...
December 27, 2017: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/29351410/hypoxia-selectively-upregulates-cation-channels-and-increases-cytosolic-ca2-in-pulmonary-but-not-coronary-arterial-smooth-muscle-cells
#16
Xi He, Shanshan Song, Ramon J Ayon, Angela Balisterieri, Stephen M Black, Ayako Makino, W Gil Wier, Wei-Jin Zang, Jason X-J Yuan
Ca2+ signaling, particularly the mechanism via store-operated Ca2+ entry (SOCE) and receptor-operated Ca2+ entry (ROCE), plays a critical role in the development of acute hypoxia-induced pulmonary vasoconstriction and chronic hypoxia-induced pulmonary hypertension. This study aimed to test the hypothesis that chronic hypoxia differentially regulates the expression of proteins that mediate SOCE and ROCE (STIM, Orai, and TRPC6) in pulmonary (PASMC) and coronary (CASMC) artery smooth muscle cells. The resting cytosolic [Ca2+] ([Ca2+]cyt) and the stored [Ca2+] in the sarcoplasmic reticulum (SR) were not different in CASMC and PASMC...
January 3, 2018: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/29351171/spinal-pkc-erk-signal-pathway-mediates-hyperalgesia-priming
#17
Wei-Hsin Chen, Ya-Ting Chang, Yong-Cyuan Chen, Sin-Jhong Cheng, Chien-Chang Chen
Chronic pain can be initiated by one or more acute stimulations to sensitize neurons into the primed state. In the primed state, the basal nociceptive thresholds of the animal are normal, but in response to another hyperalgesic stimulus, the animal develops enhanced and prolonged hyperalgesia. The exact mechanism of how primed state is formed is not completely understood. Here we showed that spinal PKC/ERK signal pathway is required for neuronal plasticity change, hyperalgesic priming formation and the development of chronic hyperalgesia using acid-induced muscle pain (AIMP) model in mice...
January 18, 2018: Pain
https://www.readbyqxmd.com/read/29351050/hidden-medical-devices-in-the-school-setting-what-the-school-nurse-needs-to-know-about-the-safe-use-of-baclofen-pumps
#18
Barbara Obst, Megan Roesler
One of the "hidden" medical devices in the school setting is the baclofen pump, which is used for the treatment of spasticity. The goals of spasticity treatment are to decrease muscle tone, deformity, and pain in order to maximize function and ease of care for both child and caregiver. The use of an intrathecal baclofen pump, often for children with cerebral palsy, spinal cord injury, brain injury, or stroke, has been effective in spasticity treatment. It is important for school nurses to be aware of the safety implications associated with this type of device...
January 1, 2018: NASN School Nurse
https://www.readbyqxmd.com/read/29351004/duchenne-muscular-dystrophy-dmd-an-updated-review-of-common-available-therapies
#19
Arash Salmaninejad, Saeed Farajzadeh Valilou, Hadi Bayat, Nader Ebadi, Abdolreza Daraei, Meysam Yousefi, Abolfazl Nesaei, Majid Mojarrad
Duchenne muscular dystrophy (DMD) is a lethal progressive pediatric muscle disorder and genetically inherited as an X-linked disease that caused by mutations in the dystrophin gene. DMD leads to progressive muscle weakness, degeneration, and wasting; finally, follows with the premature demise in affected individual's due to respiratory and/or cardiac failure typically by age of 30. For decades, scientists tried massively to find an effective therapy method, but there is no absolute cure currently for patients with DMD, nevertheless, recent advanced progressions on the treatment of DMD will be hopeful in the future...
January 19, 2018: International Journal of Neuroscience
https://www.readbyqxmd.com/read/29350554/quadriceps-neuromuscular-function-and-jump-landing-sagittal-plane-knee-biomechanics-after-anterior-cruciate-ligament-reconstruction
#20
Sarah H Ward, Troy Blackburn, Darin A Padua, Laura E Stanley, Matthew S Harkey, Brittney A Luc-Harkey, Brian G Pietrosimone
CONTEXT:   Aberrant biomechanics may affect force attenuation at the knee during dynamic activities, potentially increasing the risk of sustaining a knee injury or hastening the development of osteoarthritis after anterior cruciate ligament reconstruction (ACLR). Impaired quadriceps neuromuscular function has been hypothesized to influence the development of aberrant biomechanics. OBJECTIVE:   To determine the association between quadriceps neuromuscular function (strength, voluntary activation, and spinal-reflex and corticomotor excitability) and sagittal-plane knee biomechanics during jump landings in individuals with ACLR...
January 19, 2018: Journal of Athletic Training
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