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https://www.readbyqxmd.com/read/28098115/a-novel-fibrillin-1-mutation-in-an-egyptian-marfan-family-a-proband-showing-nephrotic-syndrome-due-to-focal-segmental-glomerulosclerosis
#1
Mohammad Al-Haggar, Ashraf Bakr, Yahya Wahba, Paul J Coucke, Fatma El-Hussini, Mona Hafez, Riham Eid, Abdel-Rahman Eid, Amr Sarhan, Ali Shaltout, Ayman Hammad, Sohier Yahia, Ahmad El-Rifaie, Dina Abdel-Hadi
Marfan syndrome (MFS), the founding member of connective tissue disorder, is an autosomal dominant disease; it is caused by a deficiency of the microfibrillar protein fibrillin-1 (FBN1) and characterized by involvement of three main systems; skeletal, ocular, and cardiovascular. More than one thousand mutations in FBN1 gene on chromosome 15 were found to cause MFS. Nephrotic syndrome (NS) had been described in very few patients with MFS being attributed to membranoproliferative glomerulonephritis secondary to infective endocarditis...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28097486/the-therapeutic-effectiveness-of-delayed-fetal-spinal-cord-tissue-transplantation-on-respiratory-function-following-mid-cervical-spinal-cord-injury
#2
Chia-Ching Lin, Sih-Rong Lai, Yu-Han Shao, Chun-Lin Chen, Kun-Ze Lee
Respiratory impairment due to damage of the spinal respiratory motoneurons and interruption of the descending drives from brainstem premotor neurons to spinal respiratory motoneurons is the leading cause of morbidity and mortality following cervical spinal cord injury. The present study was designed to evaluate the therapeutic effectiveness of delayed transplantation of fetal spinal cord (FSC) tissue on respiratory function in rats with mid-cervical spinal cord injury. Embryonic day-14 rat FSC tissue was transplanted into a C4 spinal cord hemilesion cavity in adult male rats at 1 week postinjury...
January 17, 2017: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/28097378/-molecular-pathogenesis-of-duchenne-muscular-dystrophy-related-fibrosis
#3
K Ohlendieck, D Swandulla
Progressive myofibrosis plays a key role in Duchenne muscular dystrophy. The dystrophic loss of contractile cells triggers a relatively nonspecific restructuring of the surrounding mesenchyme. The increase in connective and fatty tissue leads to muscular weakness and is therefore of critical importance for the cellular pathogenesis of muscular dystrophy. The systematic biochemical analysis of fibrosis using comparative proteomics has identified a number of extracellular matrix proteins that are indirectly involved in muscular dystrophy...
January 17, 2017: Der Pathologe
https://www.readbyqxmd.com/read/28096266/desmosomes-and-intermediate-filaments-their-consequences-for-tissue-mechanics
#4
Mechthild Hatzfeld, René Keil, Thomas M Magin
Adherens junctions (AJs) and desmosomes connect the actin and keratin filament networks of adjacent cells into a mechanical unit. Whereas AJs function in mechanosensing and in transducing mechanical forces between the plasma membrane and the actomyosin cytoskeleton, desmosomes and intermediate filaments (IFs) provide mechanical stability required to maintain tissue architecture and integrity when the tissues are exposed to mechanical stress. Desmosomes are essential for stable intercellular cohesion, whereas keratins determine cell mechanics but are not involved in generating tension...
January 17, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28095524/combination-of-collagen-barrier-membrane-with-enamel-matrix-derivative-liquid-improves-osteoblast-adhesion-and-differentiation
#5
Richard J Miron, Masako Fujioka-Kobayashi, Daniel Buser, Yufeng Zhang, Dieter D Bosshardt, Anton Sculean
PURPOSE: Collagen barrier membranes were first introduced to regenerative periodontal and oral surgery to prevent fast ingrowing soft tissues (ie, epithelium and connective tissue) into the defect space. More recent attempts have aimed at combining collagen membranes with various biologics/growth factors to speed up the healing process and improve the quality of regenerated tissues. Recently, a new formulation of enamel matrix derivative in a liquid carrier system (Osteogain) has demonstrated improved physico-chemical properties for the adsorption of enamel matrix derivative to facilitate protein adsorption to biomaterials...
January 2017: International Journal of Oral & Maxillofacial Implants
https://www.readbyqxmd.com/read/28095386/in-vitro-evaluation-of-the-cleansing-effect-of-a-monofilament-fiber-debridement-pad-compared-to-gauze-swabs
#6
Cornelia Wiegand, Kirsten Reddersen, Uta-Christina Hipler, Martin Abel, Peter Ruth, Anneke Andriessen
BACKGROUND: Removal of nonvital tissue is an accepted method to eradicate biofilms and to stimulate wound healing. Debridement using a monofilament polyester fiber pad has clinically been shown to be effective as well as pain and trauma free. METHODS: For in vitro determination of the cleansing capacity of this product compared to gauze swabs, a wound debridement model was used with glass plates coated with a bovine serum albumin solution, stained with hematoxylin...
January 18, 2017: Skin Pharmacology and Physiology
https://www.readbyqxmd.com/read/28094813/a-methylome-wide-mqtl-analysis-reveals-associations-of-methylation-sites-with-gad1-and-hdac3-snps-and-a-general-psychiatric-risk-score
#7
D M Ciuculete, A E Boström, S Voisin, H Philipps, O E Titova, M Bandstein, L Nikontovic, M J Williams, J Mwinyi, H B Schiöth
Genome-wide association studies have identified a number of single-nucleotide polymorphisms (SNPs) that are associated with psychiatric diseases. Increasing body of evidence suggests a complex connection of SNPs and the transcriptional and epigenetic regulation of gene expression, which is poorly understood. In the current study, we investigated the interplay between genetic risk variants, shifts in methylation and mRNA levels in whole blood from 223 adolescents distinguished by a risk for developing psychiatric disorders...
January 17, 2017: Translational Psychiatry
https://www.readbyqxmd.com/read/28094808/butterfly-eyespot-organiser-in-vivo-imaging-of-the-prospective-focal-cells-in-pupal-wing-tissues
#8
Mayo Iwasaki, Yoshikazu Ohno, Joji M Otaki
Butterfly wing eyespot patterns are determined in pupal tissues by organisers located at the centre of the prospective eyespots. Nevertheless, organiser cells have not been examined cytochemically in vivo, partly due to technical difficulties. Here, we directly observed organiser cells in pupal forewing epithelium via an in vivo confocal fluorescent imaging technique, using 1-h post-pupation pupae of the blue pansy butterfly, Junonia orithya. The prospective eyespot centre was indented from the plane of the ventral tissue surface...
January 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28094678/orthotic-treatment-of-positional-brachycephaly-associated-with-osteogenesis-imperfecta
#9
Carolina G Matarazzo, Gerd Schreen, Camilla D do Lago-Rizzardi, Maria Stella Peccin, Fernando Cg Pinto
Osteogenesis imperfecta is an inherited disorder of the connective tissue characterized primarily by fractures with no or small causal antecedents and extremely variable clinical presentation. The disorder requires a global and, therefore, multidisciplinary therapeutic approach that should aim, among other aspects, at the prevention and treatment of deformities resulting from osteogenesis imperfecta. Due to limitations related to bony deformities, it can be difficult to place these infants in a variety of positions that would help remediate skull deformities, so a cranial orthosis becomes the therapy of choice...
January 1, 2017: Prosthetics and Orthotics International
https://www.readbyqxmd.com/read/28094420/evaluation-of-the-optimal-plate-position-for-the-fixation-of-supraglenoid-tubercle-fractures-in-warmbloods
#10
Sina Frei, Hans Geyer, Seamus Hoey, Anton E Fuerst, Andrea S Bischofberger
OBJECTIVES: To determine scapular cortex thickness, distal scapular bone density and describe the exact suprascapular nerve course to evaluate the best plate position for the fixation of supraglenoid tubercle fractures in horses. METHODS: Twelve equine cadaveric shoulders were examined with computed tomography. Computed tomography morphometry and density measurements (Hounsfield units [HU]) of the scapula were recorded. Statistical comparisons were made between the cranial and caudal aspects of the scapula...
January 17, 2017: Veterinary and Comparative Orthopaedics and Traumatology: V.C.O.T
https://www.readbyqxmd.com/read/28094413/electrical-impedance-tomography-for-lung-ventilation-monitoring-of-the-dog
#11
Simon Gloning, Korbinian Pieper, Martin Zoellner, Andrea Meyer-Lindenberg
BACKGROUND: Electrical impedance tomography (EIT) is a radiation free technique which takes advantage of the different electrical conductivities of different tissues. Its main field of application is lung ventilation monitoring. The aim of this prospective study was to evaluate the feasibility of collecting EIT information on a sample of dogs with different thoracic shapes under clinical conditions by connecting an electrode belt without fur clipping. MATERIAL AND METHODS: Fifteen pulmonary healthy dogs were anaesthetized, positioned in sternal recumbency and ventilated in a pressure-controlled mode at three different positive end-expiratory pressure levels (PEEP) of 0, 5 and 10 cmH2O for five breaths each, with a peak inspiratory pressure of 15 cmH2O...
January 17, 2017: Tierärztliche Praxis. Ausgabe K, Kleintiere/Heimtiere
https://www.readbyqxmd.com/read/28094240/ectopic-bone-formation-by-gel-derived-bioactive-glass-poly-l-lactide-co-glycolide-composites-in-a-rabbit-muscle-model
#12
Joanna Filipowska, Katarzyna Cholewa-Kowalska, Jarosław Wieczorek, Danuta Semik, Zbigniew Dąbrowski, Maria Łączka, Anna M Osyczka
In this study we aimed to assess the in vivo osteoinductive properties of two composite scaffolds made of PLGA (poly-L-lactide-co-glycolide) and two types of gel-derived bioactive glasses, namely a high silica S2 bioactive glass (S2-PLGA composites) or high lime A2 bioactive glass (A2-PLGA composites). To achieve that, the potential of the composites to induce ectopic bone formation in a rabbit muscle has been examined along with the control PLGA scaffold. Cylinder-like scaffolds of 7  ×  3 mm (width  ×  height) were implanted into pouches created in the latissimus dorsi muscle of 18 New Zealand rabbits...
January 17, 2017: Biomedical Materials
https://www.readbyqxmd.com/read/28093577/epigenetic-research-in-neuropsychiatric-disorders-the-tissue-issue
#13
Kelly M Bakulski, Alycia Halladay, Valerie W Hu, Jonathan Mill, M Daniele Fallin
PURPOSE OF REVIEW: Evidence has linked neuropsychiatric disorders with epigenetic marks as either a biomarker of disease, biomarker of exposure, or mechanism of disease processes. Neuropsychiatric epidemiologic studies using either target brain tissue or surrogate blood tissue each have methodological challenges and distinct advantages. RECENT FINDINGS: Brain tissue studies are challenged by small sample sizes of cases and controls, incomplete phenotyping, post-mortem timing, and cellular heterogeneity, but the use of a primary disease relevant tissue is critical...
September 2016: Current Behavioral Neuroscience Reports
https://www.readbyqxmd.com/read/28093319/anti-inflammatory-effect-of-the-salvia-sclarea-l-ethanolic-extract-on-lipopolysaccharide-induced-periodontitis-in-rats
#14
Milica Kostić, Dušanka Kitić, Milica B Petrović, Tatjana Jevtović-Stojmenov, Marko Jović, Aleksandar Petrović, Slavoljub Živanović
ETHNOPHARMACOLOGICAL RELEVANCE: Salvia sclarea L., clary, is an aromatic plant traditionally used in folk medicine for the treatment of various diseases and conditions. Although it has been primarily used as a stomachic, there are data on traditional use of S. sclarea as an agent against gingivitis, stomatitis and aphthae. AIM OF THE STUDY: The aim of the study was to examine the effect of the S. sclarea ethanolic extract on the lipopolysaccharide (LPS)-induced periodontitis in rats from the immunological and histopathological standpoint...
January 13, 2017: Journal of Ethnopharmacology
https://www.readbyqxmd.com/read/28093267/interleukin-6-induces-cell-proliferation-of-clear-cell-renal-cell-carcinoma-by-suppressing-hepacam-via-the-stat3-dependent-up-regulation-of-dnmt1-or-dnmt3b
#15
Zhen Quan, Yunfeng He, Chunli Luo, Yang Xia, Yan Zhao, Nanjing Liu, Xiaohou Wu
Interleukin 6 (IL-6), a tumor promoting cytokine, has been largely implicated in the development of renal cell carcinoma (RCC). Hepatocyte cell adhesion molecule (hepaCAM) is a novel tumor suppressor, which is lost or down-regulated in many cancer types including RCC. In the present study, we intensively investigated the connection between IL-6 and hepaCAM in RCC. Our analysis of RCC tissues, adjacent tissues and paired serum samples from RCC patients revealed that IL-6 was elevated in patient serum and RCC tissue, whereas hepaCAM was completely lost or significantly down-regulated...
January 13, 2017: Cellular Signalling
https://www.readbyqxmd.com/read/28093048/myofibroblasts-are-evidence-of-chronic-tissue-microtrauma-at-the-endometrial-myometrial-junctional-zone-in-uteri-with-adenomyosis
#16
Mohamed Gamal Ibrahim, Martin Sillem, Johanna Plendl, Vito Chiantera, Jalid Sehouli, Sylvia Mechsner
BACKGROUND: Adenomyosis (AM) uteri exhibit hyperperistalsis. The latter causes a chronic tissue trauma at the endometrial-myometrial junctional zone (EMJZ). Upon tissue trauma, microdehiscences in the myometrium facilitate the translocation of basal endometrial fragments into the myometrium. There, a metaplasia (mediated by transforming growth factor β1 [TGFβ1] and connective tissue growth factor [CTGF]) occurs and AM lesions develop. The abundance of myofibroblasts in a tissue hallmarks metaplasia and points to a tissue microtrauma...
January 1, 2017: Reproductive Sciences
https://www.readbyqxmd.com/read/28092776/periosteum-tissue-engineering-in-an-orthotopic-in%C3%A2-vivo-platform
#17
J G Baldwin, F Wagner, L C Martine, B M Holzapfel, C Theodoropoulos, O Bas, F M Savi, C Werner, E M De-Juan-Pardo, D W Hutmacher
The periosteum plays a critical role in bone homeostasis and regeneration. It contains a vascular component that provides vital blood supply to the cortical bone and an osteogenic niche that acts as a source of bone-forming cells. Periosteal grafts have shown promise in the regeneration of critical size defects, however their limited availability restricts their widespread clinical application. Only a small number of tissue-engineered periosteum constructs (TEPCs) have been reported in the literature. A current challenge in the development of appropriate TEPCs is a lack of pre-clinical models in which they can reliably be evaluated...
November 20, 2016: Biomaterials
https://www.readbyqxmd.com/read/28092764/common-deregulated-gene-expression-profiles-and-morphological-changes-in-developing-zebrafish-larvae-exposed-to-environmental-relevant-high-to-low-concentrations-of-glucocorticoids
#18
Qiyu Chen, Caixia Li, Zhiyuan Gong, Eric Chun Yong Chan, Shane A Snyder, Siew Hong Lam
Synthetic glucocorticoids have been detected in environmental waters and their biological potency have raised concerns of their impact on aquatic vertebrates especially fish. In this study, developing zebrafish larvae exposed to representative glucocorticoids (dexamethasone, prednisolone and triamcinolone) at 50 pM to 50 nM from 3 h post-fertilisation to 5 days post-fertilisation were investigated. Microarray analysis identified 1255, 1531, and 2380 gene probes, which correspondingly mapped to 660, 882 and 1238 human/rodent homologs, as deregulated by dexamethasone, prednisolone and triamcinolone, respectively...
January 6, 2017: Chemosphere
https://www.readbyqxmd.com/read/28092273/in-vivo-testing-of-extracorporeal-membrane-ventilators-ila-activve%C3%A2-vs-prototype-i-lung%C3%A2
#19
Sabine Kischkel, Stefan Bergt, Beate Brock, Johan von Grönheim, Anne Herbst, Marc-Jonas Epping, Georg Matheis, Esther Novosel, Joerg Schneider, Philipp Warnke, Andreas Podbielski, Jan P Roesner, Peter I Lelkes, Brigitte Vollmar
A side-by-side comparison of the decarboxylation efficacy of two pump-driven veno-venous extracorporeal lung assist devices, i.e. a first prototype of the new miniaturized ambulatory extracorporeal membrane ventilator, I-lung® versus the commercial system iLA-activve® over a period of 72 hours in a large animal model.Fifteen German Landrace pigs were anesthetized and underwent mechanical hypoventilation to induce severe hypercapnia. Decarboxylation was accomplished by either the I-lung® or the iLA-activve® via a double lumen catheter in the jugular vein...
January 4, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28092213/cellular-and-molecular-factors-influencing-tendon-repair
#20
Sushmitha Durgam, Matthew Stewart
Tendons are complex, hierarchically organized connective tissues that transmit tensile forces between muscles and tendons. Tendon injuries are among the most common orthopedic problems with long-term disability as a frequent consequence due to prolonged healing time. Further, the repair tissue is of inferior quality, predisposing patients to high rates of recurrence following initial injury. Co-ordinated cellular processes and biological factors under the influence of mechanical loading are involved in tendon healing and our understanding of these events lags behind other musculoskeletal tissues...
January 16, 2017: Tissue Engineering. Part B, Reviews
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