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https://www.readbyqxmd.com/read/29909596/osteogenesis-imperfecta-muscle-bone-interactions-when-bi-directionally-compromised
#1
REVIEW
Charlotte L Phillips, Youngjae Jeong
PURPOSE OF REVIEW: Osteogenesis imperfecta (OI) is a hereditary connective tissue disorder of skeletal fragility and more recently muscle weakness. This review highlights our current knowledge of the impact of compromised OI muscle function on muscle-bone interactions and skeletal strength in OI. RECENT FINDINGS: The ramifications of inherent muscle weakness in OI muscle-bone interactions are just beginning to be elucidated. Studies in patients and in OI mouse models implicate altered mechanosensing, energy metabolism, mitochondrial dysfunction, and paracrine/endocrine crosstalk in the pathogenesis of OI...
June 16, 2018: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/29909553/increasing-quality-of-life-in-pulmonary-arterial-hypertension-is-there-a-role-for-nutrition
#2
REVIEW
Paulien Vinke, Suzanne M Jansen, Renger F Witkamp, Klaske van Norren
Pulmonary arterial hypertension (PAH) is a progressive disease primarily affecting the pulmonary vasculature and heart. PAH patients suffer from exercise intolerance and fatigue, negatively affecting their quality of life. This review summarizes current insights in the pathophysiological mechanisms underlying PAH. It zooms in on the potential involvement of nutritional status and micronutrient deficiencies on PAH exercise intolerance and fatigue, also summarizing the potential benefits of exercise and nutritional interventions...
June 16, 2018: Heart Failure Reviews
https://www.readbyqxmd.com/read/29909291/exercise-recovery-increases-skeletal-muscle-h-2-o-2-emission-and-mitochondrial-respiratory-capacity-following-two-weeks-of-limb-immobilization
#3
Chantal A Pileggi, Christopher P Hedges, Randall F D'Souza, Brenan R Durainayagam, James F Markworth, Anthony J R Hickey, Cameron J Mitchell, David Cameron-Smith
Extended periods of skeletal muscle disuse result in muscle atrophy. Following limb immobilization, increased mitochondrial reactive oxygen species (ROS) production may contribute to atrophy through increases in skeletal muscle protein degradation. However, the effect of skeletal muscle disuse on mitochondrial ROS production remains unclear. This study investigated the effect of immobilization, followed by two subsequent periods of restored physical activity, on mitochondrial H2 O2 emissions in adult male skeletal muscle...
June 14, 2018: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/29909101/lipoprotein-apheresis-acutely-reverses-coronary-microvascular-dysfunction-in-patients-with-severe-hypercholesterolemia
#4
Melinda D Wu, Federico Moccetti, Eran Brown, Brian P Davidson, Tamara Atkinson, J Todd Belcik, George Giraud, P Barton Duell, Sergio Fazio, Hagai Tavori, Sotirios Tsimikas, Jonathan R Lindner
OBJECTIVES: The study evaluated whether lipoprotein apheresis produces immediate changes in resting perfusion in subjects with severe hypercholesterolemia, and whether there is a difference in the response between peripheral and coronary microcirculations. BACKGROUND: Lipoprotein apheresis is used in patients with severe hypercholesterolemia to reduce plasma levels of low-density lipoprotein cholesterol. METHODS: Quantitative contrast-enhanced ultrasound perfusion imaging of the myocardium at rest and skeletal muscle at rest and during calibrated contractile exercise was performed before and immediately after lipoprotein apheresis in 8 subjects with severe hypercholesterolemia, 7 of whom had a diagnosis of familial hypercholesterolemia...
June 9, 2018: JACC. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29909017/adropin-regulates-pyruvate-dehydrogenase-in-cardiac-cells-via-a-novel-gpcr-mapk-pdk4-signaling-pathway
#5
Dharendra Thapa, Michael W Stoner, Manling Zhang, Bingxian Xie, Janet R Manning, Danielle Guimaraes, Sruti Shiva, Michael J Jurczak, Iain Scott
Mitochondria supply ~90% of the ATP required for contractile function in cardiac cells. While adult cardiomyocytes preferentially utilize fatty acids as a fuel source for oxidative phosphorylation, cardiac mitochondria can switch to other substrates when required. This change is driven in part by a combination of extracellular and intracellular signal transduction pathways that alter mitochondrial gene expression and enzymatic activity. The mechanisms by which extracellular metabolic information is conveyed to cardiac mitochondria are not currently well defined...
June 9, 2018: Redox Biology
https://www.readbyqxmd.com/read/29908653/evaluating-skeletal-muscle-electromechanical-delay-with-intramuscular-pressure
#6
Shanette A Go, William J Litchy, Loribeth Q Evertz, Kenton R Kaufman
INTRODUCTION: Intramuscular pressure (IMP) is the fluid pressure generated within skeletal muscle and directly reflects individual muscle tension. The purpose of this study was to assess the development of force, IMP, and electromyography (EMG) in the tibialis anterior (TA) muscle during ramped isometric contractions and evaluate electromechanical delay (EMD). METHODS: Force, EMG, and IMP were simultaneously measured during ramped isometric contractions in eight young, healthy human subjects...
June 8, 2018: Journal of Biomechanics
https://www.readbyqxmd.com/read/29908621/breast-and-body-contouring-for-transgender-and-gender-nonconforming-individuals
#7
REVIEW
Shane D Morrison, Stelios C Wilson, Scott W Mosser
Even after long-term hormone therapy, many transwomen still have challenges in the size and shape of their breasts and also of the contour of their trunk areas. Though distinct anthropomorphic differences in skeletal structure exist and pose limitations to an ideal result, considerable improvements can be obtained through breast augmentation and trunk feminization. There are challenges that are unique to the transfeminine chest and trunk, and important considerations for the priority of fat grafting to the buttocks and hips when grafted fat is in short supply...
July 2018: Clinics in Plastic Surgery
https://www.readbyqxmd.com/read/29908522/management-of-complete-talocrural-luxations-by-selective-talocrural-arthrodesis-using-hybrid-transarticular-external-skeletal-fixation-in-dogs
#8
Cenk Yardımcı, Taylan Önyay, Kamil S İnal, Deniz B Özbakır, Ahmet Özak
OBJECTIVE:  This article presents a novel surgical technique in the management of open complete talocrural luxations and evaluates the results, and clinical benefits with its routine clinical utilization. STUDY DESIGN:  Retrospective study. ANIMALS:  Seventeen medium- or large-breed client-owned dogs of different breed, age and sex with complete talocrural luxations and radiographic follow-up of at least 24 weeks duration. METHOD:  Selective talocrural arthrodesis was performed by using a hybrid transarticular external skeletal fixator frame...
June 16, 2018: Veterinary and Comparative Orthopaedics and Traumatology: V.C.O.T
https://www.readbyqxmd.com/read/29908035/relationship-between-tongue-pressure-and-maxillofacial-morphology-in-japanese-children-based-on-skeletal-classification
#9
Hiroyuki Kurabeishi, Ryu Tatsuo, Nezu Makoto, Fukui Kazunori
BACKGROUND: During childhood, perioral muscle function is closely associated with malocclusion. OBJECTIVE: To clarify effects of tongue function on maxillofacial morphology in children, tongue pressure and maximum lip-closing force (LCF) were measured and the relationship between perioral muscle function and maxillofacial morphology were evaluated according to skeletal classification. METHODS: Maximum tongue pressure (MTP) and swallowing tongue pressure (STP) were measured on the anterior palatine rugae in 100 children (Hellman's dental stages IIIA-IIIC) using a balloon-type tongue-pressure-measurement device...
June 16, 2018: Journal of Oral Rehabilitation
https://www.readbyqxmd.com/read/29907962/prenatal-diagnosis-of-skeletal-dysplasias-using-a-targeted-skeletal-gene-panel
#10
Xinyao Zhou, Natalie Chandler, Linbei Deng, Jia Zhou, Meizhen Yuan, Luming Sun
OBJECTIVE: This study aimed to perform an accurate and precise diagnosis for foetuses with suspected skeletal anomalies based on an incomplete and limited ultrasound phenotype. METHODS: Proband-only targeted skeletal gene panel sequencing was performed on twelve families who had foetuses with suspected skeletal anomalies based on ultrasound evaluations at a mean gestational age of 24 weeks and 3 days. The foetuses all had normal standard genetic testing yield (karyotyping and microarray)...
June 15, 2018: Prenatal Diagnosis
https://www.readbyqxmd.com/read/29907924/heat-acclimation-increases-inflammatory-and-apoptotic-responses-to-subsequent-lps-challenge-in-c2c12-myotubes
#11
Meghan G Patton, Trevor L Gillum, Mandy C Szymanski, Lacey M Gould, Claire J Lauterbach, Roger A Vaughan, Matthew R Kuennen
This work investigated the ability of a 6-day heat acclimation protocol to impart heat acclimation-mediated cross-tolerance (HACT) in C2C12 myotubes, as indicated by changes in inflammatory and apoptotic responses to subsequent lipopolysaccharide (LPS) challenge. Myotubes were incubated at 40 °C for 2 h/day over 6 days (HA) or maintained for 6 days at 37 °C (C). Following 24 h recovery, myotubes from each group received either no stimulation or 500 ng/ml LPS for 2 h (HA + LPS and C + LPS, respectively)...
June 16, 2018: Cell Stress & Chaperones
https://www.readbyqxmd.com/read/29907844/insulin-suppression-of-fatty-acid-skeletal-muscle-enzyme-activity-in-postmenopausal-women-and-improvements-in-metabolic-flexibility-and-lipoprotein-lipase-with-aerobic-exercise-and-weight-loss
#12
Alice S Ryan, Heidi K Ortmeyer
BACKGROUND: Obesity and insulin resistance are characterized by metabolic inflexibility, a condition described as an inability to switch from fat oxidation during fasting to carbohydrate oxidation during hyperinsulinemia. The purpose of this study was to examine predictors of metabolic flexibility in 103 obese (37-59% fat), sedentary (VO2 max: 19.4 ± 0.5 ml/kg/min), postmenopausal (45-76 years) women, and changes in metabolic flexibility with exercise and weight loss interventions...
June 15, 2018: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/29907690/znf341-controls-stat3-expression-and-thereby-immunocompetence
#13
Stefanie Frey-Jakobs, Julia M Hartberger, Manfred Fliegauf, Claudia Bossen, Magdalena L Wehmeyer, Johanna C Neubauer, Alla Bulashevska, Michele Proietti, Philipp Fröbel, Christina Nöltner, Linlin Yang, Jessica Rojas-Restrepo, Niko Langer, Sandra Winzer, Karin R Engelhardt, Cristina Glocker, Dietmar Pfeifer, Adi Klein, Alejandro A Schäffer, Irina Lagovsky, Idit Lachover-Roth, Vivien Béziat, Anne Puel, Jean-Laurent Casanova, Bernhard Fleckenstein, Stephan Weidinger, Sara S Kilic, Ben-Zion Garty, Amos Etzioni, Bodo Grimbacher
Signal transducer and activator of transcription 3 (STAT3) is a central regulator of immune homeostasis. STAT3 levels are strictly controlled, and STAT3 impairment contributes to several diseases including the monogenic autosomal-dominant hyper-immunoglobulin E (IgE) syndrome (AD-HIES). We investigated patients of four consanguineous families with an autosomal-recessive disorder resembling the phenotype of AD-HIES, with symptoms of immunodeficiency, recurrent infections, skeletal abnormalities, and elevated IgE...
June 15, 2018: Science Immunology
https://www.readbyqxmd.com/read/29907594/repositioning-dopamine-d2-receptor-agonist-bromocriptine-to-enhance-docetaxel-chemotherapy-and-treat-bone-metastatic-prostate-cancer
#14
Yang Yang, Kenza Mamouni, Xin Li, Yanhua Chen, Sravan Kavuri, Yuhong Du, Haian Fu, Omer Kucuk, Daqing Wu
Docetaxel resistance remains a major obstacle in the treatment of prostate cancer (PCa) bone metastasis. In this study, we demonstrate that the dopamine D2 receptor (DRD2) agonist bromocriptine effectively enhances docetaxel efficacy and suppresses skeletal growth of PCa in preclinical models. DRD2 is ubiquitously expressed in PCa cell lines, and DRD2 is significantly reduced in PCa tissues with high Gleason score. Bromocriptine has weak to moderate cytotoxicity in PCa cells, but effectively induces cell cycle arrest...
June 15, 2018: Molecular Cancer Therapeutics
https://www.readbyqxmd.com/read/29907477/successful-treatment-of-normokalemic-periodic-paralysis-with-hydrochlorothiazide
#15
Yuichi Akaba, Satoru Takahashi, Yoshiaki Sasaki, Hiroki Kajino
BACKGROUND: Periodic paralysis (PP) is an autosomal dominant muscle disorder characterized by periodic muscle weakness attacks associated with serum potassium level variations. It is classified into hypokalemic (hypoKPP), hyperkalemic (hyperKPP), and normokalemic (normoKPP) forms based on the ictal serum potassium level. HyperKPP and normoKPP are caused by mutations of the same gene SCN4A, the gene encoding the skeletal muscle voltage-gated sodium channel. Prophylactic treatment with thiazide diuretics is highly effective in preventing attacks in hyperKPP...
June 12, 2018: Brain & Development
https://www.readbyqxmd.com/read/29907353/determinants-of-skeletal-muscle-protein-turnover-following-severe-burn-trauma-in-children
#16
Ioannis Malagaris, David N Herndon, Efstathia Polychronopoulou, Victoria G Rontoyanni, Clark R Andersen, Oscar E Suman, Craig Porter, Labros S Sidossis
BACKGROUND & AIMS: Burns remain the fifth cause of non-fatal pediatric injuries globally, with muscle cachexia being a hallmark of the stress response to burns. Burn-induced muscle wasting is associated with morbidity, yet the determinants of muscle protein catabolism in response to burn trauma remains unclear. Our objective was to determine the effect of patient and injury characteristics on muscle protein kinetics in burn patients. METHODS: This retrospective, observational study was performed using protein kinetic data from pediatric patients who had severe burns (>30% of the total body surface area burned) and underwent cross-limb stable isotope infusions between 1999 and 2008 as part of prospective clinical trials...
June 4, 2018: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29907249/heritability-of-muscle-mass-in-korean-parent-offspring-pairs-in-the-fifth-korean-national-health-and-nutrition-examination-survey-knhanes-v
#17
Ju-Young You, Yun-Jee Kim, Woo-Young Shin, Na-Yeon Kim, Soo Hyun Cho, Jung-Ha Kim
OBJECTIVES: Decreased muscle mass is known to be associated with several serious medical conditions. We analyzed the Fifth Korean National Health and Nutrition Examination Survey (KNHANES V, 2010-2011) to estimate the heritability of muscle mass in Korean parent-offspring pairs. STUDY DESIGN: Cross-sectional. MAIN OUTCOME MEASURES: A total of 1233 parents (average age 57.67 ± 8.50 years) and 917 offspring (average age 29.10 ± 7.57 years) from 743 families were included in the analysis...
August 2018: Maturitas
https://www.readbyqxmd.com/read/29906781/structural-optimization-and-amorphous-calcium-phosphate-mineralization-in-sensory-setae-of-a-terrestrial-crustacean-isopoda-oniscidea
#18
Miloš Vittori, Vesna Srot, Birgit Bussmann, Felicitas Predel, Peter A van Aken, Jasna Štrus
Terrestrial isopods possess large sensory setae on their walking legs. Increased fracture resistance of these elongated structures is of crucial importance, making the exoskeleton forming the setae an interesting durable material that may inspire biomimetic designs. We studied the cuticle of the sensory setae with analytical electron microscopy in order to gain detailed insights into its structure and composition at the nanometer scale and identify features that increase the fracture resistance of these minute skeletal elements...
June 9, 2018: Micron: the International Research and Review Journal for Microscopy
https://www.readbyqxmd.com/read/29906668/elongated-odontoid-process-in-late-holocene-skeletal-remains-from-b6-archaeological-site-mendoza-argentina
#19
J A Suby, P Novellino, G Da Peña, C D Pandiani
The odontoid process of the axis can be affected by congenital or acquired pathologies. While abnormalities such as os odontoideum, agenesis, and fractures are reported in archaeological remains, the abnormality of an elongated length of the odontoid process has not been described in the paleopathological literature. The aim of this paper is to evaluate two individuals with elongated odontoid processes from a skeletal assemblage from the B6 archaeological site (Mendoza, Argentina), and to discuss the possible etiologies of the condition, with particular attention given to the relation to trauma and Crowned Dens Syndrome (CDS), a condition characterized by the ossification of ligaments of the odontoid process of the second cervical vertebra...
June 12, 2018: International Journal of Paleopathology
https://www.readbyqxmd.com/read/29906337/retained-differentiation-capacity-of-human-skeletal-muscle-satellite-cells-from-spinal-cord-injured-individuals
#20
Mladen Savikj, Maxwell A Ruby, Emil Kostovski, Per O Iversen, Juleen R Zierath, Anna Krook, Ulrika Widegren
Despite the well-known role of satellite cells in skeletal muscle plasticity, the effect of spinal cord injury on their function in humans remains unknown. We determined whether spinal cord injury affects the intrinsic ability of satellite cells to differentiate and produce metabolically healthy myotubes. We obtained vastus lateralis biopsies from eight spinal cord-injured and six able-bodied individuals. Satellite cells were isolated, grown and differentiated in vitro. Gene expression was measured by quantitative PCR...
June 2018: Physiological Reports
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