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Collagen VI

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https://www.readbyqxmd.com/read/28087121/upper-extremity-outcome-measures-for-collagen-vi-related-myopathy-and-lama2-related-muscular-dystrophy
#1
Roxanna M Bendixen, Jocelyn Butrum, Mina S Jain, Rebecca Parks, Bonnie Hodsdon, Carmel Nichols, Michelle Hsia, Leslie Nelson, Katherine C Keller, Michelle McGuire, Jeffrey S Elliott, Melody M Linton, Irene C Arveson, Fatou Tounkara, Ruhi Vasavada, Elizabeth Harnett, Monal Punjabi, Sandra Donkervoort, Jahannaz Dastgir, Meganne E Leach, Anne Rutkowski, Melissa Waite, James Collins, Carsten G Bönnemann, Katherine G Meilleur
Congenital muscular dystrophy (CMD) comprises a rare group of genetic muscle diseases that present at birth or early during infancy. Two common subtypes of CMD are collagen VI-related muscular dystrophy (COL6-RD) and laminin alpha 2-related dystrophy (LAMA2-RD). Traditional outcome measures in CMD include gross motor and mobility assessments, yet significant motor declines underscore the need for valid upper extremity motor assessments as a clinical endpoint. This study validated a battery of upper extremity measures in these two CMD subtypes for future clinical trials...
December 5, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28075471/alterations-in-splenic-function-and-gene-expression-in-mice-with-depressive-like-behavior-induced-by-exposure-to-corticosterone
#2
Heqin Zhan, Feng Huang, Fulin Yan, Zhenghang Zhao, Jixia Zhang, Taizhen Cui, Fan Yang, Guangfan Hai, Xiaoman Jia, Yongji Shi
Depressed patients present with increased cortisol levels and attenuated immune responses. However, little is known about the association between depression and the spleen, as this is the largest peripheral immune organ. In this study, we examined alterations in splenic function and gene expression in mice with depressive-like behavior, well as the expression of certain proteins in related pathways. A mouse model of depression was established with the use of corticosterone. Splenic function and histopathology were assessed using Wright and H&E staining...
January 5, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28071602/microgrooved-topographical-surface-directs-tenogenic-lineage-specific-differentiation-of-mouse-tendon-derived-stem-cells
#3
Yuan Shi, Kaili Zhou, Wenjie Zhang, Zhiyong Zhang, Guangdong Zhou, Yilin Cao, Wei Liu
Tendon derived stem cells (TDSCs) are the endogenous cell source for tenocyte turnover and tendon functional maintenance. They are also the important cell source for tendon engineering and regeneration. In addition, TDSCs also play an important role in tendinopathy via their non-tenogenic lineage differentiation. It has been well demonstrated that cell shape could determine mesenchymal stem cell (MSC) lineage differentiation. In this study, a parallel microgrooved polydimethylsiloxane (PDMS) membrane (10 µm groove width and 3 µm depth) was employed to investigate the role of cell elongation via this particular topographic surface in directing murine TDSC (mTDSC) lineage differentiation...
January 10, 2017: Biomedical Materials
https://www.readbyqxmd.com/read/28054640/honokiol-as-a-specific-collagen-receptor-glycoprotein-vi-antagonist-on-human-platelets-functional-ex-vivo-and-in-vivo-studies
#4
Tzu-Yin Lee, Chao-Chien Chang, Wan-Jung Lu, Ting-Lin Yen, Kuan-Hung Lin, Pitchairaj Geraldine, Jiun-Yi Li, Joen-Rong Sheu
Honokiol, derived from Magnolia officinalis, has various pharmacological properties. Platelet activation plays a critical role in cardiovascular diseases. Honokiol has been reported to inhibit collagen-stimulated rabbit platelet aggregation. However, detailed further studies on the characteristics and functional activity of honokiol in platelet activation are relatively lacking. In the present study, honokiol specifically inhibited platelet aggregation and Ca(+2) ion mobilization stimulated with collagen or convulxin, an agonist of glycoprotein (GP) VI, but not with aggretin, an agonist of integrin α2β1...
January 5, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28043812/muscle-weakness-and-fibrosis-due-to-cell-autonomous-and-non-cell-autonomous-events-in-collagen-vi-deficient-congenital-muscular-dystrophy
#5
Satoru Noguchi, Megumu Ogawa, May Christine Malicdan, Ikuya Nonaka, Ichizo Nishino
Congenital muscular dystrophies with collagen VI deficiency are inherited muscle disorders with a broad spectrum of clinical presentation and are caused by mutations in one of COL6A1-3 genes. Muscle pathology is characterized by fiber size variation and increased interstitial fibrosis and adipogenesis. In this study, we define critical events that contribute to muscle weakness and fibrosis in a mouse model with collagen VI deficiency. The Col6a1(GT/GT) mice develop non-progressive weakness from younger age, accompanied by stunted muscle growth due to reduced IGF-1 signaling activity...
February 2017: EBioMedicine
https://www.readbyqxmd.com/read/28009413/role-of-dextran-in-maintaining-adhesive-and-stiffness-properties-of-prestripped-dmek-lenticules
#6
Mohit Parekh, Alessandro Ruzza, Rosa Di Mundo, Stefano Ferrari, Giuseppina Recchia, Hossein Elbadawy, Giuseppe Carbone, Diego Ponzin
PURPOSE: To investigate the adhesive and stiffness properties of prestripped Descemet membrane endothelial keratoplasty (DMEK) lenticules in different preservation conditions (with and without dextran). METHODS: The study included 3 conditions: (C1) tissues collected from tissue culture media (TCM), stripped and preserved in TCM; (C2) tissues collected from transport media (TM) (TCM supplemented with 6% dextran T-500), stripped and preserved in TM; and (C3) tissues collected from TCM, stripped and preserved in TM...
November 25, 2016: European Journal of Ophthalmology
https://www.readbyqxmd.com/read/28004756/alteration-of-platelet-gpvi-signaling-in-st-elevation-myocardial-infarction-patients-demonstrated-by-a-combination-of-proteomic-biochemical-and-functional-approaches
#7
Paula Vélez, Raymundo Ocaranza-Sánchez, Diego López-Otero, Lilian Grigorian-Shamagian, Isaac Rosa, Esteban Guitián, José María García-Acuña, José Ramón González-Juanatey, Ángel García
The platelet-specific collagen receptor glycoprotein VI (GPVI) is critical for the formation of arterial thrombosis in vivo. We analyzed GPVI-activated platelets from ST-elevation myocardial infarction (STEMI) patients and matched stable coronary artery disease (SCAD) controls in order to provide novel clues on the degree of involvement of GPVI signaling in the acute event. Firstly, platelets were isolated from systemic venous blood and activated with the GPVI specific agonist CRP (collagen-related peptide)...
December 22, 2016: Scientific Reports
https://www.readbyqxmd.com/read/28002907/tgf-%C3%AE-1-differentially-modulates-the-collagen-vi-%C3%AE-5-and-%C3%AE-6-chains-in-human-tendon-cultures
#8
P Sabatelli, F Sardone, F Traina, L Merlini, S Santi, R Wagener, C Faldini
Collagen VI is a microfibrillar collagen with a potential regulatory role in tendon repair mechanism. We studied the expression of collagen VI α5 and α6 chains in normal human tendon fibroblast cultures, both under basal condition and in response to TGF-β1, a potent regulator of tendon healing. Under basal condition, we found that the α5 chain was expressed, although to a lesser extent with respect to the α3 chain; in contrast, the α6 chain was absent. The treatment with TGFβ1 induced an opposite effect on the expression of the α5 and α6 chains; in fact, while the α5 chain was dramatically reduced, the α6 chain was induced and released in the culture medium...
October 2016: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/27999100/platelet-hyperreactivity-in-diabetes-focus-on-gpvi-signaling-are-useful-drugs-already-available
#9
Jane F Arthur, Karin Jandeleit-Dahm, Robert K Andrews
Adults with diabetes are 2-4 times more likely to suffer from heart disease or ischemic stroke than adults without diabetes, yet standard antiplatelet therapy, which is the cornerstone for primary and secondary prevention of cardiovascular disease, fails in many patients with diabetes. Three independent but often interrelated variables that contribute to platelet hyperreactivity-high blood glucose, oxidative stress, and elevated vascular shear forces-coexist in patients with diabetes, creating a perilous concurrence of risk factors for cardiovascular events...
January 2017: Diabetes
https://www.readbyqxmd.com/read/27996269/novel-glycoprotein-vi-antagonists-as-antithrombotics-synthesis-biological-evaluation-and-molecular-modeling-studies-on-2-3-disubstituted-tetrahydropyrido-3-4-b-indoles
#10
Shome S Bhunia, Ankita Misra, Imran A Khan, Stuti Gaur, Manish Jain, Surendra Singh, Aaruni Saxena, Thomas Hohlfield, Madhu Dikshit, Anil K Saxena
The development of small molecule inhibitors targeting GPVI has promising therapeutic role, as they inhibit arterial thrombosis with limited risk of bleeding. Among the compounds showing in vivo antithrombotic activity, the most active compound 6b (ED50 = 28.36 μmol/kg po in mice) showed improved inhibition for collagen (IC50 = 6.7 μM), CRP-XL (IC50 = 53.5 μM), and convulxin (CVX) (IC50 = 5.7 μM) mediated platelet aggregation as compared to losartan (LOS) (collagen, IC50 = 10.4 μM; CRP-XL, IC50 = 158 μM; CVX, IC50 = 11 μM) than any of its enantiomers S (6c) (collagen, IC50 = 25...
December 20, 2016: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/27992950/hierarchical-organization-of-the-hemostatic-response-to-penetrating-injuries-in-the-mouse-macrovasculature
#11
John D Welsh, Izmarie Poventud-Fuentes, Sara Sampietro, Scott L Diamond, Timothy J Stalker, Lawrence F Brass
BACKGROUND: Intravital studies performed in the mouse microcirculation show that hemostatic thrombi formed after penetrating injuries develop a characteristic architecture in which a core of fully-activated, densely-packed platelets is overlaid with a shell of less activated platelets. OBJECTIVE: Large differences in hemodynamics and vessel wall biology distinguish arteries from arterioles. Here we asked whether these differences affect the hemostatic response and alter the impact of anticoagulants and antiplatelet agents...
December 19, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27991501/the-immunohistochemical-and-urodynamic-evaluation-towards-the-collagen-coated-and-non-coated-polypropylene-meshes-implanted-in-the-pelvic-wall-of-the-rats
#12
Tsia-Shu Lo, Yi-Hao Lin, Faridah Mohd Yusoff, Hsiao-Chien Chu, Wu-Chiao Hsieh, Ma Clarissa Uy-Patrimonio
Our aim is to study the inflammatory response towards the collagen-coated and non-coated polypropylene meshes in rats and the urodynamic investigation post-operatively. Forty-two female Sprague Dawley were divided into 7 groups of 6 rats; Control, Day 7 and 30 for Sham, Avaulta Plus (MPC), Perigee (MP). UDS were taken at days 7 and 30. Mesh with the vagina and bladder wall was removed and sent for immunohistochemical examination. Results showed intense inflammatory reaction on day 7 in the study groups which decreased on day 30...
December 19, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27956360/defining-the-hierarchical-organisation-of-collagen-vi-microfibrils-at-nanometre-to-micrometre-length-scales
#13
Alan R F Godwin, Tobias Starborg, Michael J Sherratt, Alan M Roseman, Clair Baldock
: Extracellular matrix microfibrils are critical components of connective tissues with a wide range of mechanical and cellular signalling functions. Collagen VI is a heteromeric network-forming collagen which is expressed in tissues such as skin, lung, blood vessels and articular cartilage where it anchors cells into the matrix allowing for transduction of biochemical and mechanical signals. It is not understood how collagen VI is arranged into microfibrils or how these microfibrils are arranged into tissues...
December 10, 2016: Acta Biomaterialia
https://www.readbyqxmd.com/read/27932089/clinical-and-neuroimaging-findings-in-two-brothers-with-limb-girdle-muscular-dystrophy-due-to-lama2-mutations
#14
Elizabeth Harris, Meriel McEntagart, Ana Topf, Hanns Lochmüller, Kate Bushby, Caroline Sewry, Volker Straub
Recessive mutations in LAMA2 commonly cause congenital muscular dystrophy (MDC1A) and, rarely, limb girdle muscular dystrophy (LGMD). We report 2 brothers who presented in adulthood with LGMD due to novel mutations in LAMA2 identified by whole exome sequencing (WES). Muscle biopsy more than 30 years ago demonstrated dystrophic changes but was not available for immunoanalysis. Muscle MRI demonstrated involvement of peripheral muscle with internal sparing classically seen in collagen-VI related disorders. Extensive genetic testing, including COL6A1/2/3, was performed prior to WES...
November 3, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27929413/exosome-proteome-of-u-87mg-glioblastoma-cells
#15
Sohyun Chun, Seunghyun Ahn, Chang-Hwan Yeom, Seyeon Park
Exosomes are small membrane vesicles between 30 and 100 nm in diameter secreted by many cell types, and are associated with a wide range of physiological and/or pathological processes. Exosomes containing proteins, lipids, mRNA, and microRNA contribute to cell-to-cell communication and cell-to-environment regulation, however, their biological functions are not yet fully understood. In this report, exosomes in the glioblastoma cell line, U-87MG, were isolated and the proteome was investigated. In addition, exosome proteome changes in U-87MG cells exposed to a low temperature were investigated to elucidate whether the exosome proteome could respond to an external stimulus...
December 6, 2016: Biology
https://www.readbyqxmd.com/read/27924510/global-protein-expression-analysis-of-molecular-markers-of-ds-1-47-a-component-of-implantation-promoting-traditional-chinese-medicine
#16
Yan-Ling Li, Xiao-Yan Zhang, Yu Leng, Yan-Li Wu, Jing Li, Yun-Xia Wu
This study investigated the molecular markers of DS-1-47, a component of an implantation- promoting traditional Chinese medicine consisting of Astragalus mongholicus, Atractylodes macrocephala, Scutellaria baicalensis and Dipsacales, in an attempt to clarify the molecular mechanism and action targets of DS-1-47. Controlled ovarian stimulation (COS) method was used to establish the implantation dysfunction models of mice. Animals were divided into normal pregnant group, COS model group and DS-1-47 group. Laser capture microdissection-double dimensional electrophoresis-mass spectrum (LCM-DE-MS) was used to analyze the uterine protein molecules that were possibly involved in the promotion of implantation...
December 2016: Journal of Huazhong University of Science and Technology. Medical Sciences
https://www.readbyqxmd.com/read/27885904/the-effect-of-p2y12-inhibition-on-platelet-activation-assessed-with-aggregation-and-flow-cytometry-based-assays
#17
Tesse C Leunissen, Peter Paul Wisman, Thijs C van Holten, Philip G de Groot, Suzanne J Korporaal, Arnold C Koekman, Frans L Moll, Martin Teraa, Marianne C Verhaar, Gert Jan de Borst, Rolf T Urbanus, Mark Roest
Patients on P2Y12 inhibitors may still develop thrombosis or bleeding complications. Tailored antiplatelet therapy, based on platelet reactivity testing, might reduce these complications. Several tests have been used, but failed to show a benefit of tailored antiplatelet therapy. This could be due to the narrowness of current platelet reactivity tests, which are limited to analysis of platelet aggregation after stimulation of the adenosine diphosphate (ADP)-pathway. However, the response to ADP does not necessarily reflect the effect of P2Y12 inhibition on platelet function in vivo...
November 25, 2016: Platelets
https://www.readbyqxmd.com/read/27884442/the-induction-of-the-collagen-capsule-synthesis-by-trichinella-spiralis-is-closely-related-to-protease-activated-receptor-2
#18
Mi Kyung Park, Min Kyoung Cho, Shin Ae Kang, Bo Young Kim, Hak Sun Yu
The muscle-stage larvae of the parasite Trichinella spiralis have the ability to survive within host muscle tissue by virtue of the formation a nurse cell-parasite complex, which is surrounded by collagen. The formation of the complex is initiated by excretory-secretory (ES) proteins produced by the parasite. To determine the mechanisms underlying collagen capsule formation, we investigated the expression levels of several types of collagen genes and TGF-βI signaling-related genes (Smad2 and Smad3) in muscle cells...
October 30, 2016: Veterinary Parasitology
https://www.readbyqxmd.com/read/27870875/a-novel-organ-culture-model-to-quantify-collagen-remodeling-in-tree-shrew-sclera
#19
Sarah Baldivia, Alexander Levy, Shylaja Hegde, Stijn J A Aper, Maarten Merkx, Rafael Grytz
Increasing evidence suggests that unknown collagen remodeling mechanisms in the sclera underlie myopia development. We are proposing a novel organ culture system in combination with two-photon fluorescence imaging to quantify collagen remodeling at the tissue- and lamella-level. Tree shrew scleral shells were cultured up to 7 days in serum-free media and cellular viability was investigated under: (i) minimal tissue manipulations; (ii) removal of intraocular tissues; gluing the eye to a washer using (iii) 50 μL and (iv) 200 μL of cyanoacrylate adhesive; (v) supplementing media with Ham's F-12 Nutrient Mixture; and (vi) culturing eyes subjected to 15 mmHg intraocular pressure in our new bioreactor...
2016: PloS One
https://www.readbyqxmd.com/read/27854213/bethlem-myopathy-phenotypes-and-follow-up-description-of-8-patients-at-the-mildest-end-of-the-spectrum
#20
Simao Cruz, Sebastian Figueroa-Bonaparte, Eduard Gallardo, Alix de Becdelièvre, Corine Gartioux, Valérie Allamand, Patricia Piñol, Maria Angels Rodriguez Garcia, Cecilia Jiménez-Mallebriera, Jaume Llauger, Lidia González-Rodríguez, Elena Cortes-Vicente, Isabel Illa, Jordi Díaz-Manera
The classical phenotypes of collagen VI-associated myopathies are well described. Little is known, however, about the progression of patients at the mildest end of the clinical spectrum. In this report, we describe the clinical findings and the results of MRI, muscle biopsy, collagen VI expression in cultured skin fibroblasts and genetic tests of a series of patients with Bethlem myopathy. Our series highlights the existence of mild presentations of this disorder that progresses only slightly and can easily be overlooked...
May 27, 2016: Journal of Neuromuscular Diseases
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