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Collagen VI

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https://www.readbyqxmd.com/read/28918041/gapmer-antisense-oligonucleotides-suppress-the-mutant-allele-of-col6a3-and-restore-functional-protein-in-ullrich-muscular-dystrophy
#1
Elena Marrosu, Pierpaolo Ala, Francesco Muntoni, Haiyan Zhou
Dominant-negative mutations in the genes that encode the three major α chains of collagen type VI, COL6A1, COL6A2, and COL6A3, account for more than 50% of Ullrich congenital muscular dystrophy patients and nearly all Bethlem myopathy patients. Gapmer antisense oligonucleotides (AONs) are usually used for gene silencing by stimulating RNA cleavage through the recruitment of an endogenous endonuclease known as RNase H to cleave the RNA strand of a DNA-RNA duplex. In this study, we exploited the application of the allele-specific silencing approach by gapmer AON as a potential therapy for Collagen-VI-related congenital muscular dystrophy (COL6-CMD)...
September 15, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28888895/regulation-of-platelet-activation-and-thrombus-formation-by-reactive-oxygen-species
#2
Jianlin Qiao, Jane F Arthur, Elizabeth E Gardiner, Robert K Andrews, Lingyu Zeng, Kailin Xu
Reactive oxygen species (ROS) are generated within activated platelets and play an important role in regulating platelet responses to collagen and collagen-mediated thrombus formation. As a major collagen receptor, platelet-specific glycoprotein (GP)VI is a member of the immunoglobulin (Ig) superfamily, with two extracellular Ig domains, a mucin domain, a transmembrane domain and a cytoplasmic tail. GPVI forms a functional complex with the Fc receptor γ-chain (FcRγ) that, following receptor dimerization, signals via an intracellular immunoreceptor tyrosine-based activation motif (ITAM), leading to rapid activation of Src family kinase signaling pathways...
September 7, 2017: Redox Biology
https://www.readbyqxmd.com/read/28878675/inhibition-of-collagen-induced-platelet-aggregation-by-the-secobutanolide-secolincomolide-a-from-lindera-obtusiloba-blume
#3
Sang-Hyuk Jung, Joo-Hui Han, Hyun-Soo Park, Jung-Jin Lee, Seo Young Yang, Young Ho Kim, Kyung-Sun Heo, Chang-Seon Myung
Atherothrombosis is one of the main underlying cause of cardiovascular diseases. In addition to treating atherothrombosis with antithrombotic agents, there is growing interest in the role of natural food products and biologically active ingredients for the prevention and treatment of cardiovascular diseases. This study aimed to investigate the effect of secolincomolide A (3) isolated from Lindera obtusiloba Blume on platelet activity and identify possible signaling pathways. In our study, the antiplatelet activities of 3 were measured by collagen-induced platelet aggregation and serotonin secretion in freshly isolated rabbit platelets...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28877744/exome-sequences-versus-sequential-gene-testing-in-the-uk-highly-specialised-service-for-limb-girdle-muscular-dystrophy
#4
Elizabeth Harris, Ana Topf, Rita Barresi, Judith Hudson, Helen Powell, James Tellez, Debbie Hicks, Anna Porter, Marta Bertoli, Teresinha Evangelista, Chiara Marini-Betollo, Ólafur Magnússon, Monkol Lek, Daniel MacArthur, Kate Bushby, Hanns Lochmüller, Volker Straub
BACKGROUND: Limb girdle muscular dystrophies are a group of rare and genetically heterogeneous diseases that share proximal weakness as a common feature; however they are often lacking very specific phenotypic features to allow an accurate differential diagnosis based on the clinical signs only, limiting the diagnostic rate using phenotype driven genetic testing. Next generation sequencing provides an opportunity to obtain molecular diagnoses for undiagnosed patients, as well as identifying novel genetic causes of muscle diseases...
September 6, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28874524/direct-dating-of-neanderthal-remains-from-the-site-of-vindija-cave-and-implications-for-the-middle-to-upper-paleolithic-transition
#5
Thibaut Devièse, Ivor Karavanić, Daniel Comeskey, Cara Kubiak, Petra Korlević, Mateja Hajdinjak, Siniša Radović, Noemi Procopio, Michael Buckley, Svante Pääbo, Tom Higham
Previous dating of the Vi-207 and Vi-208 Neanderthal remains from Vindija Cave (Croatia) led to the suggestion that Neanderthals survived there as recently as 28,000-29,000 B.P. Subsequent dating yielded older dates, interpreted as ages of at least ∼32,500 B.P. We have redated these same specimens using an approach based on the extraction of the amino acid hydroxyproline, using preparative high-performance liquid chromatography (Prep-HPLC). This method is more efficient in eliminating modern contamination in the bone collagen...
September 5, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28870220/the-type-vi-adenylyl-cyclase-protects-cardiomyocytes-from-%C3%AE-adrenergic-stress-by-a-pka-stat3-dependent-pathway
#6
Yu-Shuo Wu, Chien-Chang Chen, Chen-Li Chien, Hsing-Lin Lai, Si-Tse Jiang, Yong-Cyuan Chen, Lin-Ping Lai, Wei-Fan Hsiao, Wen-Pin Chen, Yijuang Chern
BACKGROUND: The type VI adenylyl cyclase (AC6) is a main contributor of cAMP production in the heart. The amino acid (aa) sequence of AC6 is highly homologous to that of another major cardiac adenylyl cyclase, AC5, except for its N-terminus (AC6-N, aa 1-86). Activation of AC6, rather than AC5, produces cardioprotective effects against heart failure, while the underlying mechanism remains to be unveiled. Using an AC6-null (AC6(-/-)) mouse and a knockin mouse with AC6-N deletion (AC6 (ΔN/ΔN)), we aimed to investigate the cardioprotective mechanism of AC6 in the heart...
September 4, 2017: Journal of Biomedical Science
https://www.readbyqxmd.com/read/28855423/plant-based-food-cyanidin-3-glucoside-modulates-human-platelet-glycoprotein-vi-signaling-and-inhibits-platelet-activation-and-thrombus-formation
#7
Yanling Yao, Yanqiu Chen, Reheman Adili, Thomas McKeown, Pingguo Chen, Guangheng Zhu, Dan Li, Wenhua Ling, Heyu Ni, Yan Yang
Background: Platelets play an important role in hemostasis, thrombosis, and atherosclerosis. Glycoprotein VI (GPVI) is a major platelet receptor that interacts with exposed collagen on injured vessel walls. Our previous studies have shown that anthocyanins (a type of natural plant pigment) attenuate platelet function; however, whether anthocyanins affect collagen-induced GPVI signaling remains unknown.Objective: The objective of this study was to explore the effects of cyanidin-3-glucoside (Cy-3-g, one of the major bioactive compounds in anthocyanins) on platelet activation and thrombosis and the GPVI signaling pathway...
August 30, 2017: Journal of Nutrition
https://www.readbyqxmd.com/read/28841424/mechanical-induction-of-dentin-like-differentiation-by-adult-mouse-bone-marrow-stromal-cells-using-compressive-scaffolds
#8
Basma Hashmi, Tadanori Mammoto, James Weaver, Thomas Ferrante, Amanda Jiang, Elisabeth Jiang, Juani Feliz, Donald E Ingber
Tooth formation during embryogenesis is controlled through a complex interplay between mechanical and chemical cues. We have previously shown that physical cell compaction of dental mesenchyme cells during mesenchymal condensation is responsible for triggering odontogenic differentiation during embryogenesis, and that expression of Collagen VI stabilizes this induction. In addition, we have shown that synthetic polymer scaffolds that artificially induce cell compaction can induce embryonic mandible mesenchymal cells to initiate tooth differentiation both in vitro and in vivo...
August 17, 2017: Stem Cell Research
https://www.readbyqxmd.com/read/28831785/clinical-pathologic-and-genetic-features-of-collagen-vi-related-myopathy-in-korea
#9
Jung Hwan Lee, Ha Young Shin, Hyung Jun Park, Se Hoon Kim, Seung Min Kim, Young Chul Choi
BACKGROUND AND PURPOSE: Mutations in collagen VI-related genes (COL6A1, COL6A2, and COL6A3) cause Bethlem myopathy (BM) and Ullrich congenital muscular dystrophy (UCMD). These were previously believed to be separate disease entities, but they are now both classified as collagen VI-related myopathies, which cover a broad clinical spectrum. We aimed to analyze the clinical, pathologic, and genetic characteristics of patients with collagen VI-related myopathy in Korea. METHODS: We reviewed the clinical, pathologic, and genetic features in 22 patients with collagen VI-related myopathy from 13 families, as confirmed by genetic analysis of collagen VI-related genes...
August 1, 2017: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/28821957/co-culture-of-chondrons-and-mesenchymal-stromal-cells-reduces-the-loss-of-collagen-vi-and-improves-extracellular-matrix-production
#10
H A Owida, T De Las Heras Ruiz, A Dhillon, Y Yang, N J Kuiper
Adult articular chondrocytes are surrounded by a pericellular matrix (PCM) to form a chondron. The PCM is rich in hyaluronan, proteoglycans, and collagen II, and it is the exclusive location of collagen VI in articular cartilage. Collagen VI anchors the chondrocyte to the PCM. It has been suggested that co-culture of chondrons with mesenchymal stromal cells (MSCs) might enhance extracellular matrix (ECM) production. This co-culture study investigates whether MSCs help to preserve the PCM and increase ECM production...
August 19, 2017: Histochemistry and Cell Biology
https://www.readbyqxmd.com/read/28760337/somatic-mosaicism-represents-an-underestimated-event-underlying-collagen-6-related-disorders
#11
Adele D'Amico, Fabiana Fattori, Giorgio Tasca, Stefania Petrini, Francesca Gualandi, Alessandro Bruselles, Valentina D'Oria, Margherita Verardo, Rosalba Carrozzo, Marcello Niceta, Bjarne Udd, Alessandra Ferlini, Marco Tartaglia, Enrico Bertini
BACKGROUND: Collagen VI-related disorders (COL6-RD) are a group of heterogenous muscular diseases due to mutations in the COL6A1, COL6A2 and COL6A3 genes, encoding for collagen VI, a critical component of the extracellular matrix. Ullrich congenital muscle disorder and Bethlem myopathy represent the ends of a clinical spectrum that includes intermediate phenotypes of variable severity. UCMD are caused by recessive loss of function mutations or de-novo dominant-negative mutations. The intermediate phenotype and BM are more commonly caused by dominantly acting mutations, and less commonly by recessive mutations...
July 22, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28755659/authentication-of-collagen-vi-antibodies
#12
Jamie Endicott, Paul Holden, Jamie Fitzgerald
BACKGROUND: Collagen VI is a ubiquitously-expressed macromolecule that forms unique microfibrillar assemblies in the extracellular matrix. Mutations in the COL6A1, COL6A2 and COL6A3 genes result in congenital muscular dystrophy, arguing that collagen is critical for skeletal muscle development and function. Antibodies against collagen VI are important clinical and diagnostic tools in muscular dystrophy. They are used to confirm genetic findings by detecting abnormalities in the distribution, organization and overall levels of collagen VI in cells and tissues isolated from patients...
July 29, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28751543/adpase-cd39-fused-to-glycoprotein-vi-fc-boosts-local-antithrombotic-effects-at-vascular-lesions
#13
Heidrun Degen, Oliver Borst, Melanie Ziegler, Ann-Katrin Mojica Munoz, Janina Jamasbi, Britta Walker, Silvia Göbel, Julia Fassbender, Kristin Adler, Richard Brandl, Götz Münch, Reinhard Lorenz, Wolfgang Siess, Meinrad Gawaz, Martin Ungerer
BACKGROUND: GPVI (Glycoprotein VI) is the essential platelet collagen receptor in atherothrombosis. Dimeric GPVI-Fc (Revacept) binds to GPVI binding sites on plaque collagen. As expected, it did not increase bleeding in clinical studies. GPVI-Fc is a potent inhibitor of atherosclerotic plaque-induced platelet aggregation at high shear flow, but its inhibition at low shear flow is limited. We sought to increase the platelet inhibitory potential by fusing GPVI-Fc to the ectonucleotidase CD39 (fusion protein GPVI-CD39), which inhibits local ADP accumulation at vascular plaques, and thus to create a lesion-directed dual antiplatelet therapy that is expected to lack systemic bleeding risks...
July 27, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28750654/different-therapeutic-effects-of-cells-derived-from-human-amniotic-membrane-on-premature-ovarian-aging-depend-on-distinct-cellular-biological-characteristics
#14
Chenyue Ding, Hong Li, Yun Wang, Fuxin Wang, Huihua Wu, Rulei Chen, Jinghuan Lv, Wei Wang, Boxian Huang
BACKGROUND: Many reports have shown that various kinds of stem cells have the ability to recover premature ovarian aging (POA) function. Transplantation of human amniotic epithelial cells (hAECs) improves ovarian function damaged by chemotherapy in a mice model. Understanding of how to evaluate the distinct effects of adult stem cells in curing POA and how to choose stem cells in clinical application is lacking. METHODS: To build a different degrees of POA model, mice were administered different doses of cyclophosphamide: light dose (70 mg/kg, 2 weeks), medium dose (70 mg/kg, 1 week; 120 mg/kg, 1 week), and high dose (120 mg/kg, 2 weeks)...
July 27, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/28739510/differential-proteomics-reveals-s100-a11-as-a-key-factor-in-aldosterone-induced-collagen-expression-in-human-cardiac-fibroblasts
#15
Ernesto Martínez-Martínez, Jaime Ibarrola, Mercedes Lachén-Montes, Amaya Fernández-Celis, Frederic Jaisser, Enrique Santamaría, Joaquín Fernández-Irigoyen, Natalia López-Andrés
Aldosterone (Aldo) could induce cardiac fibrosis, a hallmark of heart disease. Aldo direct effects on collagen production in cardiac fibroblasts remain controversial. Our aim is to characterize changes in the proteome of adult human cardiac fibroblasts treated with Aldo to identify new proteins altered that might be new therapeutic targets in cardiovascular diseases. Aldo increased collagens expressions in human cardiac fibroblasts. Complementary, using a quantitative proteomic approach, 30 proteins were found differentially expressed between control and Aldo-treated cardiac fibroblasts...
July 22, 2017: Journal of Proteomics
https://www.readbyqxmd.com/read/28736303/the-good-and-the-bad-collagens-of-fibrosis-their-role-in-signaling-and-organ-function
#16
M A Karsdal, S H Nielsen, D J Leeming, L L Langholm, M J Nielsen, T Manon-Jensen, A Siebuhr, N S Gudmann, S Rønnow, J M Sand, S J Daniels, J H Mortensen, D Schuppan
Usually the dense extracellular structure in fibrotic tissues is described as extracellular matrix (ECM) or simply as collagen. However, fibrosis is not just fibrosis, which is already exemplified by the variant morphological characteristics of fibrosis due to viral versus cholestatic, autoimmune or toxic liver injury, with reticular, chicken wire and bridging fibrosis. Importantly, the overall composition of the ECM, especially the relative amounts of the many types of collagens, which represent the most abundant ECM molecules and which centrally modulate cellular functions and physiological processes, changes dramatically during fibrosis progression...
July 20, 2017: Advanced Drug Delivery Reviews
https://www.readbyqxmd.com/read/28727490/comparison-of-the-release-of-micrornas-and-extracellular-vesicles-from-platelets-in-response-to-different-agonists
#17
Ashley R Ambrose, Mohammed A Alsahli, Sameer A Kurmani, Alison H Goodall
On activation platelets release microRNAs and extracellular vesicles (EV) into circulation. The release of EV from platelets has been shown to be dependent on the agonist; in this study, we investigated whether the microRNA profile or EV released from platelets was also agonist specific. Washed platelets from healthy subjects were maximally stimulated with agonists specific for the receptors for collagen (Glycoprotein VI (GPVI)), thrombin (PAR1/PAR4), or ADP (P2Y1/P2Y12) with/without inhibiting secondary mediators, using aspirin to block cyclooxygenase-1 and apyrase to remove ADP...
July 20, 2017: Platelets
https://www.readbyqxmd.com/read/28725908/cyst-like-lesions-at-chondro-osseous-junction
#18
Zijun Zhang, Jeffrey Beckett, Lew Schon
This study described and histologically characterized a cyst-like lesion (CLL) at the chondro-osseous junction. Rat knees (n = 12), with or without excessive running-induced osteoarthritis (OA), were used for counting the incidence, morphological measurements, immunohistochemistry of the CLL. A typical CLL, appearing as a void space in the matrix, was located on the tidemark at the chondro-osseous junction. The content of the CLL included types II and VI collagen, proteoglycans but not intact chondrocytes. At least one CLL was found in 5/6 osteoarthritic knees and only 2/6 in the non-osteoarthritic knees...
July 19, 2017: Calcified Tissue International
https://www.readbyqxmd.com/read/28714570/human-osteoarthritic-chondrons-outnumber-patient-and-joint-matched-chondrocytes-in-hydrogel-culture-future-application-in-autologous-cell-based-oa-cartilage-repair
#19
Miriam Rothdiener, Tatiana Uynuk-Ool, Norbert Südkamp, Matthias Aurich, Alan J Grodzinsky, Bodo Kurz, Bernd Rolauffs
Autologous chondrocyte implantation (ACI) is used in 34-60% for osteoarthritic (OA) cartilage defects, although ACI is neither recommended nor designed for OA. Envisioning a hydrogel-based ACI for OA that uses chondrons instead of classically used chondrocytes, we hypothesized that human OA-chondrons may outperform OA-chondrocytes. We compared patient- and joint surface-matched human OA-chondrons vs. OA-chondrocytes cultured for the first time in a hydrogel, using a self-assembling peptide system. We determined yield, viability, cell numbers, mRNA expression, GAPDH mRNA enzyme activity, collagen II synthesis (CPII) and degradation (C2C), and sGAG...
July 17, 2017: Journal of Tissue Engineering and Regenerative Medicine
https://www.readbyqxmd.com/read/28713908/comparison-of-gene-expression-profiles-between-dental-pulp-and-periodontal-ligament-tissues-in-humans
#20
Ai-Xiu Gong, Jing-Han Zhang, Jing Li, Jun Wu, Lin Wang, Deng-Shun Miao
There are anatomical and functional differences between human dental pulp (DP) and periodontal ligament (PDL). However, the molecular biological differences and function of these tissues are poorly understood. In the present study, we employed a cDNA microarray array to screen for differentially expressed genes (DEGs) between human DP and PDL tissues, and used the online software WebGestalt to perform the functional analysis of the DEGs. In addition, the STRING database and KEGG pathway analysis were applied for interaction network and pathway analysis of the DEGs...
September 2017: International Journal of Molecular Medicine
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