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Collagen VI

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https://www.readbyqxmd.com/read/28182637/comprehensive-target-capture-next-generation-sequencing-as-a-second-tier-diagnostic-approach-for-congenital-muscular-dystrophy-in-taiwan
#1
Wen-Chen Liang, Xia Tian, Chung-Yee Yuo, Wan-Zi Chen, Tsu-Min Kan, Yi-Ning Su, Ichizo Nishino, Lee-Jun C Wong, Yuh-Jyh Jong
PURPOSE: Congenital muscular dystrophy (CMD) is a heterogeneous disease entity. The detailed clinical manifestation and causative gene for each subgroup of CMD are quite variable. This study aims to analyze the phenotypes and genotypes of Taiwanese patients with CMD as the epidemiology of CMD varies among populations and has been scantly described in Asia. METHODS: A total of 48 patients suspected to have CMD were screened and categorized by histochemistry and immunohistochemistry studies...
2017: PloS One
https://www.readbyqxmd.com/read/28178939/corneal-vesicles-accumulate-collagen-vi-associated-with-tissue-remodeling-in-apolipoprotein-a-i-deficiency-a-case-report
#2
Hiroyuki Namba, Mari Narumi, Shinji Susa, Rintaro Ohe, Takeo Kato, Mitsunori Yamakawa, Hidetoshi Yamashita
BACKGROUND: Apo A-I deficiency clinically shows low serum levels of HDL cholesterol and corneal opacity at a young age. Histopathological evaluations of affected corneas are not enough, and the mechanism of corneal opacity is still unclear. CASE PRESENTATION: A 61-year-old woman suffered from blurred vision with a corneal opacity. She had significantly reduced serum levels of high-density lipoprotein cholesterol and Apo A-I, stenosis of the coronary arteries, and ischemic heart failure...
February 8, 2017: BMC Ophthalmology
https://www.readbyqxmd.com/read/28164654/expression-of-platelet-collagen-receptor-glycoprotein-vi-in-coronary-heart-disease-interaction-between-platelets-and-endothelial-cells
#3
Jian Shi, Ning Zhu, Jiwen Wang, Yu Wang, Zhaohong Geng, Suya Gao, Zhongyan Li
BACKGROUND: 1) To investigate the expression of platelet collagen receptor glycoprotein VI (GPVI) on the surface of platelets in patients with coronary heart disease (CHD). 2) To investigate the expression of intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) on the surface of endothelial cells after exposure to subjects' platelets. METHODS: Platelets were extracted from blood samples of 97 subjects including 43 with acute coronary syndrome (ACS), 21 with stable angina pectoris (SAP), and 33 serving as controls in which CHD was excluded...
July 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28163696/vasoinhibin-an-n-terminal-prolactin-fragment-directly-inhibits-cardiac-angiogenesis-in-three-dimensional-heart-culture
#4
Ryojun Nakajima, Eri Nakamura, Toshio Harigaya
Vasoinhibins (Vi) are fragments of the growth hormone/prolactin (PRL) family and have antiangiogenic functions in many species. It is considered that Vi derived from PRL are involved in the pathogenesis of peripartum cardiomyopathy (PPCM). However, the pathogenic mechanism of PPCM, as well as heart angiogenesis, is not yet clear. Therefore, the aim of the present study is to clarify whether Vi act directly on angiogenesis inhibition in heart blood vessels. Endothelial cell viability was decreased by Vi treatment in a culture experiment...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28155721/computer-prediction-of-paratope-on-antithrombotic-antibody-10b12-and-epitope-on-platelet-glycoprotein-vi-via-molecular-dynamics-simulation
#5
Wenping Liu, Guangjian Liu, Huiyun Zhou, Xiang Fang, Ying Fang, Jianhua Wu
BACKGROUND: Interaction between immunoglobulin-like receptor glycoprotein VI (GPVI) and collagen plays a central role in platelet activation and sequent firm adhesion. Of various antithrombotic agents targeting GPVI, antibody 10B12 is of great potential to block the GPVI-collagen interaction, but less is known about 10B12 paratope and GPVI epitope. METHODS: Along the pathway in the computer strategy presented in our previous work, the 10B12/GPVI complex model was constructed through homology modeling and rigid-body docking, and the molecular dynamics (MD) simulation was used to detect the paratope residues on 10B12 and their partners on GPVI...
December 28, 2016: Biomedical Engineering Online
https://www.readbyqxmd.com/read/28148498/assessment-of-roles-for-the-rho-specific-guanine-nucleotide-dissociation-inhibitor-rhogdi-ly-gdi-in-platelet-function-a-spatial-systems-approach
#6
Anh T P Ngo, Marisa L D Thierheimer, Özgün Babur, Anne D Rocheleau, Tao Huang, Jiaqing Pang, Rachel A Rigg, Annachiara Mitrugno, Dan Theodorescu, Julja Burchard, Xiaolin Nan, Emek Demir, Owen J T McCarty, Joseph E Aslan
Upon activation at sites of vascular injury, platelets undergo morphological alterations essential to hemostasis via cytoskeletal reorganizations driven by the Rho GTPases Rac1, Cdc42 and RhoA. Here we investigate roles for Rho-specific guanine nucleotide dissociation inhibitor proteins (RhoGDIs) in platelet function. We find that platelets express two RhoGDI family members, RhoGDI and Ly-GDI. While RhoGDI localizes throughout platelets in a granule-like manner, Ly-GDI shows an asymmetric, polarized localization that largely overlaps with Rac1 and Cdc42 as well as microtubules and protein kinase C (PKC) in platelets adherent to fibrinogen...
February 1, 2017: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/28143939/blockade-to-pathological-remodeling-of-infarcted-heart-tissue-using-a-porcupine-antagonist
#7
Jesung Moon, Huanyu Zhou, Li-Shu Zhang, Wei Tan, Ying Liu, Shanrong Zhang, Lorraine K Morlock, Xiaoping Bao, Sean P Palecek, Jian Q Feng, Noelle S Williams, James F Amatruda, Eric N Olson, Rhonda Bassel-Duby, Lawrence Lum
The secreted Wnt signaling molecules are essential to the coordination of cell-fate decision making in multicellular organisms. In adult animals, the secreted Wnt proteins are critical for tissue regeneration and frequently contribute to cancer. Small molecules that disable the Wnt acyltransferase Porcupine (Porcn) are candidate anticancer agents in clinical testing. Here we have systematically assessed the effects of the Porcn inhibitor (WNT-974) on the regeneration of several tissue types to identify potentially unwanted chemical effects that could limit the therapeutic utility of such agents...
February 14, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28121998/rheumatic-heart-disease-and-myxomatous-degeneration-differences-and-similarities-of-valve-damage-resulting-from-autoimmune-reactions-and-matrix-disorganization
#8
Carlo de Oliveira Martins, Lea Demarchi, Frederico Moraes Ferreira, Pablo Maria Alberto Pomerantzeff, Carlos Brandao, Roney Orismar Sampaio, Guilherme Sobreira Spina, Jorge Kalil, Edecio Cunha-Neto, Luiza Guilherme
Autoimmune inflammatory reactions leading to rheumatic fever (RF) and rheumatic heart disease (RHD) result from untreated Streptococcus pyogenes throat infections in individuals who exhibit genetic susceptibility. Immune effector mechanisms have been described that lead to heart tissue damage culminating in mitral and aortic valve dysfunctions. In myxomatous valve degeneration (MXD), the mitral valve is also damaged due to non-inflammatory mechanisms. Both diseases are characterized by structural valve disarray and a previous proteomic analysis of them has disclosed a distinct profile of matrix/structural proteins differentially expressed...
2017: PloS One
https://www.readbyqxmd.com/read/28103932/biomarkers-of-extracellular-matrix-turnover-are-associated-with-emphysema-and-eosinophilic-bronchitis-in-copd
#9
Asger Reinstrup Bihlet, Morten Asser Karsdal, Jannie Marie Bülow Sand, Diana Julie Leeming, Mustimbo Roberts, Wendy White, Russell Bowler
BACKGROUND: Chronic obstructive pulmonary disease (COPD) is characterized by airflow obstruction and loss of lung tissue mainly consisting of extracellular matrix (ECM). Three of the main ECM components are type I collagen, the main constituent in the interstitial matrix, type VI collagen, and elastin, the signature protein of the lungs. During pathological remodeling driven by inflammatory cells and proteases, fragments of these proteins are released into the bloodstream, where they may serve as biomarkers for disease phenotypes...
January 19, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28097933/inflammatory-myopathy-in-a-patient-with-collagen-vi-mutations
#10
R Papa, C Fiorillo, C Malattia, F Minoia, R Caorsi, S Assereto, M Iacomino, M Savarese, V Nigro, C Bruno, C Minetti, P Picco
No abstract text is available yet for this article.
January 18, 2017: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/28097378/-molecular-pathogenesis-of-duchenne-muscular-dystrophy-related-fibrosis
#11
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/28087121/upper-extremity-outcome-measures-for-collagen-vi-related-myopathy-and-lama2-related-muscular-dystrophy
#12
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
#13
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...
February 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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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