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Collagen VI

L Alberto Llacua, Arjan Hoek, Bart J de Haan, Paul de Vos
Collagens are the most abundant fibrous protein in the human body and constitute the main structural element of the extracellular matrix. It provides mechanical and physiological support for cells. In the pancreas, collagen VI content is more than double that of collagen I or IV. It is a major component of the islet-exocrine interface and could be involved in islet-cell survival. To test the impact of collagen VI on human encapsulated pancreatic islets-cells, we tested the effects of exogenous collagen type VI on in vitro functional survival of alginate encapsulated human islet-cells...
March 9, 2018: Islets
Marjaana Ojalill, Pekka Rappu, Elina Siljamäki, Pekka Taimen, Peter Boström, Jyrki Heino
BACKGROUND: The composition and organization of extracellular matrix (ECM) are important regulators of cell behavior. In particular in the prostate, this central role of the ECM is further stressed by the fact that several potential markers of prostate stem cells are matrix receptors. METHODS: We established 12 fibroblastic cell lines from cancerous and non-cancerous areas of six prostates and allowed the cells to produce ECM under cell culture conditions. We also performed a proteome wide analysis of the ECM components by mass spectrometry...
March 9, 2018: Prostate
Emma Åhrman, Oskar Hallgren, Lars Malmström, Ulf Hedström, Anders Malmström, Leif Bjermer, Xiao-Hong Zhou, Gunilla Westergren-Thorsson, Johan Malmström
Remodeling of the extracellular matrix (ECM) is a common feature in lung diseases such as chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF). Here, we applied a sequential tissue extraction strategy to describe disease-specific remodeling of human lung tissue in disease, using end-stages of COPD and IPF. Our strategy was based on quantitative comparison of the disease proteomes, with specific focus on the matrisome, using data-independent acquisition and targeted data analysis (SWATH-MS)...
March 1, 2018: Journal of Proteomics
Nathan T Montgomery, Keith D Zientek, Elena N Pokidysheva, Hans Peter Bächinger
Type IV collagen is a major component of the basement membrane and interacts with numerous other basement membrane proteins.  Many of these interactions are poorly characterized.  Type IV collagen is abundantly post-translationally modified with 3-hydroxyproline (3-Hyp), but 3-Hyp's biochemical role in type IV collagen's interactions with other proteins is not well established.  In this work, we present binding data consistent with a major role of 3-Hyp in interactions of collagen IV with glycoprotein VI and nidogens 1 and 2...
February 28, 2018: Journal of Biological Chemistry
Christina Lund Kidholm, Hans Christian Beck, Julie Bukh Madsen, Nikolai Bjødstrup Palstrøm, Jes Sanddal Lindholt, Lars Melholt Rasmussen
OBJECTIVE: The pathogenesis of abdominal aortic aneurysms (AAA) involves a disturbed balance of breakdown and buildup of arterial proteins. We envision that individuals with AAA carry generalized arterial protein alterations either because of effects of genetically or environmental AAA risk factors or because of compensatory changes due to signaling molecules released from the affected aneurysmal tissue. APPROACH: Protein extraction and quantitative proteome analysis by LC-MS/MS (liquid chromatography-mass spectrometry) was done on individual samples from the internal mammary artery from 11 individuals with AAA and 33 sex- and age-matched controls without AAA...
2018: PloS One
Alexander Eckersley, Kieran T Mellody, Suzanne M Pilkington, Christopher Em Griffiths, Rachel E B Watson, Ronan O'Cualain, Clair Baldock, David Knight, Michael J Sherratt
Elastic fibres comprising fibrillin microfibrils and elastin are present in many tissues, including the skin, lung, and arteries where they confer elasticity and resilience. Although fibrillin microfibrils play distinct and tissue-specific functional roles, it is unclear whether their ultrastructure and composition differ between elastin-rich (skin) and elastin-poor (ciliary body and zonule) organs or after in vitro synthesis by cultured cells. Here, we used atomic force microscopy, which revealed that the bead morphology of fibrillin microfibrils isolated from the human eye differs from those isolated from the skin...
February 16, 2018: Journal of Biological Chemistry
Manuela Cervelli, Alessia Leonetti, Guglielmo Duranti, Stefania Sabatini, Roberta Ceci, Paolo Mariottini
Skeletal muscle comprises approximately 40% of the total body mass. Preserving muscle health and function is essential for the entire body in order to counteract chronic diseases such as type II diabetes, cardiovascular diseases, and cancer. Prolonged physical inactivity, particularly among the elderly, causes muscle atrophy, a pathological state with adverse outcomes such as poor quality of life, physical disability, and high mortality. In murine skeletal muscle C2C12 cells, increased expression of the spermine oxidase (SMOX) enzyme has been found during cell differentiation...
February 14, 2018: Medical Sciences: Open Access Journal
Peixi Liu, Yaying Song, Yingjie Zhou, Yingjun Liu, Tianming Qiu, Qingzhu An, Jianping Song, Peiliang Li, Yuan Shi, Sichen Li, Kai Quan, Guo-Yuan Yang, Wei Zhu
BACKGROUND/AIMS: Cerebral aneurysm growth is characterized by continuous structural weakness of local smooth muscle cells, though the mechanism is unclear. In this study, we examine protein changes in cerebral aneurysm and human brain vascular smooth muscle cells after cyclic mechanical stretch. We further explore the relationship between the smooth muscle cell changes and reductions in the levels of collagen types IV and VI. METHODS: Saccular cerebral aneurysms (n=10) were collected, and temporal artery samples were used as controls...
February 7, 2018: Cellular Physiology and Biochemistry
Jane A O'Halloran, Eimear Dunne, Willard Tinago, Stephanie Denieffe, Dermot Kenny, Patrick W G Mallon
OBJECTIVES: Altered platelet function has been proposed as an underlying mechanism to explain increased risk of myocardial infarction in people living with HIV associated with use of the nucleoside reverse transcriptase inhibitor abacavir (ABC). We aimed to examine changes in platelet biomarkers in people living with HIV switching from ABC. METHODS: In a prospective, 48-week substudy of virally suppressed HIV-1-positive subjects randomized to remain on ABC/lamivudine (ABC/3TC) or switch to tenofovir disoproxil fumarate/emtricitabine, we measured soluble glycoprotein VI (sGPVI), soluble P-selectin, soluble CD40 ligand and von Willebrand factor in plasma collected over time and assessed differences using mixed effect models...
February 12, 2018: AIDS
Signe H Nielsen, Naja D Mygind, Marie M Michelsen, Daria F Bechsgaard, Hannah E Suhrs, Federica Genovese, Henning B Nielsen, Susanne Brix, Morten Karsdal, Eva Prescott, Jens Kastrup
Aim Collagens are major cardiac extracellular matrix components, known to be actively remodelled and accumulated during diffuse myocardial fibrosis. We evaluated whether accelerated collagen turnover described by neo-epitope biomarkers reflecting collagen formation and degradation separates patients with diffuse myocardial fibrosis from asymptomatic controls. Methods and results Seventy-one women with angina pectoris without significant coronary artery disease assessed by invasive coronary angiogram were included...
January 1, 2018: European Journal of Preventive Cardiology
Y Fan, A Liu, C Wei, H Yang, X Chang, S Wang, Y Yuan, C Bonnemann, Q Wu, X Wu, H Xiong
Collagen VI-related myopathy, caused by pathogenic variants in the genes encoding collagen VI, represents a clinical continuum from Ullrich congenital muscular dystrophy (UCMD) to Bethlem myopathy (BM). Clinical data of 60 probands and their family members were collected and muscle biopsies of 26 patients were analyzed. COL6A1, COL6A2 and COL6A3 exons were analyzed by direct sequencing or next generation sequencing (NGS). Sixty patients were characterized by delayed motor milestones, muscle weakness, skin and joint changes with forty UCMD and twenty BM...
February 8, 2018: Clinical Genetics
Jennifer O Buitrago, Kapil D Patel, Ahmed El-Fiqi, Jung-Hwan Lee, Banani Kundu, Hae-Hyoung Lee, Hae-Won Kim
Cell encapsulating hydrogels with tunable mechanical and biological properties are of special importance for cell delivery and tissue engineering. Silk fibroin and collagen, two typical important biological proteins, are considered potential as cell culture hydrogels. However, both have been used individually, with limited properties (e.g., collagen has poor mechanical properties and cell-mediated shrinkage, and silk fibroin lacks cell adhesion motifs). Therefore, the combination of them is considered to achieve improved mechanical and biological properties with respect to individual hydrogels...
February 1, 2018: Acta Biomaterialia
Sandra Murphy, Margit Zweyer, Michael Henry, Paula Meleady, Rustam R Mundegar, Dieter Swandulla, Kay Ohlendieck
The highly progressive neuromuscular disorder dystrophinopathy is triggered by primary abnormalities in the Dmd gene, which causes cytoskeletal instability and loss of sarcolemmal integrity. Comparative organellar proteomics was employed to identify sarcolemma-associated proteins with an altered concentration in dystrophic muscle tissue from the mdx-4cv mouse model of dystrophinopathy. A lectin agglutination method was used to prepare a sarcolemma-enriched fraction and resulted in the identification of 190 significantly changed protein species...
February 1, 2018: Journal of Proteomics
Soo Yeon Kim, Woo Joong Kim, Hyuna Kim, Sun Ah Choi, Jin Sook Lee, Anna Cho, Se Song Jang, Byung Chan Lim, Ki Joong Kim, Jong-Il Kim, Si Houn Hahn, Jong-Hee Chae
INTRODUCTION: We aimed to analyze the clinical and genetic characteristics of collagen VI-related myopathy. METHODS: We analyzed the clinical course and mutation spectrum in patients with collagen VI gene mutations among our congenital muscular dystrophy cohort. RESULTS: Among 24 patients with mutations in collagen VI coding genes, 13 (54.2%) were categorized as Ullrich type, and 11 (45.8%) as non-Ullrich type. Congenital orthopedic problems were similarly observed in both types, yet multiple joint contractures were found only in the Ullrich type...
February 6, 2018: Muscle & Nerve
Netanya S Utay, Douglas W Kitch, Eunice Yeh, Carl J Fichtenbaum, Michael M Lederman, Jacob D Estes, Claire Deleage, Clara Magyar, Scott D Nelson, Karen L Klingman, Barbara Bastow, Amneris E Luque, Grace A McComsey, Daniel C Douek, Judith S Currier, Jordan E Lake
Background: Fibrosis in lymph nodes may limit CD4+ T-cell recovery and lymph node and adipose tissue fibrosis may contribute to inflammation and comorbidities despite antiretroviral therapy (ART). We hypothesized that the angiotensin receptor blocker and PPAR-γ agonist telmisartan would decrease lymph node or adipose tissue fibrosis in treated HIV-1 infection. Methods: In this 48-week, randomized-controlled trial, adults continued suppressive ART and received telmisartan or no drug...
February 1, 2018: Journal of Infectious Diseases
Qiang Zhao, Ke Wu, Nannan Li, Zhengmei Li, Fenglin Jin
Myocardial infarction (MI) is a heart disease with high morbidity and mortality rates, thus it is critical to identify genes that serve roles during its pathogenesis. The objective of the present study was to identify potentially relevant genes during the progression of the disease. Blood samples from patients with MI and normal controls (n=3/group) were obtained, the RNA was extracted and cDNA libraries were established. RNA sequencing (RNA-seq) was performed on a HiSeq 2500 platform and fragments per kilobase of exon per million fragments mapped was utilized to calculate the gene expression value following preprocessing of the RNA-seq data...
February 2018: Experimental and Therapeutic Medicine
Patrick Münzer, Sophie Mittelstädt, Sascha Geue, Mailin-Christin Manke, Britta Walker-Allgaier, Florian Lang, Meinrad Gawaz, Oliver Borst
Platelet aggregation, dense granule secretion and thrombus formation are dependent on sphingolipids like ceramide and sphingosine as well as sphingosine-1 phosphate. Sphingosine/ceramide metabolism involves ceramide synthases and ceramidases. However, the role of ceramide synthase and ceramidase in the regulation of platelet function remained ill-defined. The present study determined transmission light aggregometry, employed luciferase based ATP release measurements and studied in vitro thrombus formation under high arterial shear rates in order to define the impact of pharmacological inhibition of serine palmitoyltransferase, ceramide synthase and ceramidase on platelet function...
February 12, 2018: Biochemical and Biophysical Research Communications
Mariam Ebrahim, Janina Jamasbi, Kristin Adler, Remco T A Megens, Yacine M'Bengue, Xavier Blanchet, Kerstin Uhland, Martin Ungerer, Richard Brandl, Christian Weber, Natalie Elia, Reinhard Lorenz, Götz Münch, Wolfgang Siess
Platelet glycoprotein VI (GPVI) acts as a decisive collagen receptor in atherothrombosis. Besides collagen, injured atherosclerotic plaques expose tissue factor (TF) that triggers fibrin formation. Two recent studies reported that platelet GPVI also functions as fibrin receptor, which would importantly widen the mode of action of GPVI-targeted antithrombotic drugs. We studied the binding of two GPVI fusion proteins to fibrin under static and arterial flow conditions. Fibrin was prepared from purified fibrinogen or generated more physiologically from endogenous fibrinogen by coagulating plasma with thrombin...
February 2018: Thrombosis and Haemostasis
Monique Y Rennie, Stephanie Stovall, James P Carson, Michael Danilchik, Kent L Thornburg, Sandra Rugonyi
Blood flow is critical for normal cardiac development. Hemodynamic stimuli outside of normal ranges can lead to overt cardiac defects, but how early heart tissue remodels in response to altered hemodynamics is poorly understood. This study investigated changes in tissue collagen in response to hemodynamic overload in the chicken embryonic heart outflow tract (OFT) during tubular heart stages (HH18 to HH24, ~24 h). A suture tied around the OFT at HH18 was tightened to constrict the lumen for ~24 h (constriction range at HH24: 15-60%)...
December 20, 2017: Journal of Cardiovascular Development and Disease
Phil-Sun Oh, Eun-Mi Kim, Minjoo Kim, In Sun Kim, Yeon-Hee Han, SeokTae Lim, Myung-Hee Sohn, Myoung-Hwan Ko, Hwan-Jeong Jeong
Previous studies have reported that 450 nm blue light emitting diode (BLED) induces apoptosis through a mitochondria-mediated pathway in cancer cells and reduces the early stage tumor growth. The present study was performed to determine the effects of BLED-irradiated cell metabolites on cell injury. Our results showed that conditioned medium (CM) from cells irradiated with low-dose BLED (LCM) inhibited apoptosis and increased cell survival. Cell protection-related proteins were identified in cell metabolites of CM and LCM using 2-DE and MALDI-TOF analysis...
January 19, 2018: Photochemistry and Photobiology
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