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Collagen VI

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https://www.readbyqxmd.com/read/29774307/bethlem-myopathy-in-a-portuguese-patient-case-report
#1
Ana Inês Martins, Cristin Maarque, Jorge Pinto-Basto, Luis Negrão
Mutations of the encoding genes of collagen VI (COL6A1, COL6A2 and COL6A3 ), are responsible for two classical phenotypes (with a wide range of severity), the Ullrich congenital muscular dystrophy (UCMD) and the Bethlem myopathy (BM). We present a male patient of 49 years old, with symptoms of muscle weakness beginning in childhood and of very slowly progression. At the age of 42, the neurological examination revealed proximal lower limb muscle weakness and contractures of fingers flexors muscles, positive Gowers manoeuvre and a waddling gait...
September 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/29765702/is-radiographic-progression-in-radiographic-axial-spondyloarthritis-related-to-matrix-metalloproteinase-degradation-of-extracellular-matrix
#2
Anne Sofie Siebuhr, Desirée van der Heijde, Anne-C Bay-Jensen, Morten Asser Karsdal, Robert Landewé, Astrid van Tubergen, Sofia Ramiro
Background: Radiographic axial spondyloarthritis (r-axSpA) is associated with extracellular matrix (ECM) remodelling of affected tissues. We investigated whether there was a relationship between biomarkers of ECM remodelling and 2-year radiographic progression in r-axSpA. Methods: Patients from the Outcome in Ankylosing Spondylitis International Study (OASIS) were included if they had serum, clinical and spinal radiographic assessments available at baseline and 2 years later...
2018: RMD Open
https://www.readbyqxmd.com/read/29764467/association-of-il17rc-and-col6a1-genetic-polymorphisms-with-susceptibility-to-ossification-of-the-thoracic-posterior-longitudinal-ligament-in-chinese-patients
#3
Peng Wang, Xiao Liu, Bin Zhu, Yunlong Ma, Lei Yong, Ze Teng, Chen Liang, Guanping He, Xiaoguang Liu
BACKGROUND: In our previous whole-genome sequencing study of 30 unrelated northern Chinese Han patients, we identified six single nucleotide polymorphisms (SNPs) in the interleukin 17 receptor C (IL17RC) and collagen type VI α1 chain (COL6A1) genes that were potentially associated with thoracic ossification of the posterior longitudinal ligament (T-OPLL). To determine whether these six SNPs are associated with susceptibility to T-OPLL in the northern Chinese Han population, we performed a case-control association study to confirm specific susceptible loci in the expanded samples...
May 15, 2018: Journal of Orthopaedic Surgery and Research
https://www.readbyqxmd.com/read/29760565/genome-wide-association-pathway-analysis-to-identify-candidate-single-nucleotide-polymorphisms-and-molecular-pathways-associated-with-tp53-expression-status-in-hbv-related-hepatocellular-carcinoma
#4
Xiwen Liao, Long Yu, Xiaoguang Liu, Chuangye Han, Tingdong Yu, Wei Qin, Chengkun Yang, Guangzhi Zhu, Hao Su, Tao Peng
Background: The aim of this investigation was to identify candidate single nucleotide polymorphisms (SNPs) and molecular pathways associated with tumor protein p53 (TP53) expression status in hepatitis B virus (HBV)-related hepatocellular carcinoma (HCC), clarify their potential mechanisms, and generate SNP-to-gene to pathway hypothesis. Materials and methods: Identify candidate Causal SNPs and Pathways (ICSNPathway) was used to perform pathway analysis based on the results of our previous genome-wide association study of TP53 expression status in 387 HBV-related HCC patients...
2018: Cancer Management and Research
https://www.readbyqxmd.com/read/29752552/collagen-vi-is-required-for-the-structural-and-functional-integrity-of-the-neuromuscular-junction
#5
Matilde Cescon, Ilaria Gregorio, Nane Eiber, Doriana Borgia, Aurora Fusto, Patrizia Sabatelli, Michele Scorzeto, Aram Megighian, Elena Pegoraro, Said Hashemolhosseini, Paolo Bonaldo
The synaptic cleft of the neuromuscular junction (NMJ) consists of a highly specialized extracellular matrix (ECM) involved in synapse maturation, in the juxtaposition of pre- to post-synaptic areas, and in ensuring proper synaptic transmission. Key components of synaptic ECM, such as collagen IV, perlecan and biglycan, are binding partners of one of the most abundant ECM protein of skeletal muscle, collagen VI (ColVI), previously never linked to NMJ. Here, we demonstrate that ColVI is itself a component of this specialized ECM and that it is required for the structural and functional integrity of NMJs...
May 11, 2018: Acta Neuropathologica
https://www.readbyqxmd.com/read/29730712/homing-genes-expression-in-fucosyltransferase-vi-treated-umbilical-cord-blood-cd133-cells-which-expanded-on-protein-coated-nanoscaffolds
#6
Amir Atashi, Maryam Islami, Yousef Mortazavi, Masoud Soleimani
Umbilical cord blood (UCB)-derived hematopoietic stem cells (HSCs) are considered because of their self-renewing, differentiating, proliferating, and readily available properties. Moreover, HSCs' homing to the hematopoietic microenvironment is an important step in their transplantation process. But low content of progenitor cells in one unit of UCB and defect in the bone marrow (BM) homing limit their applications. Hence, we decided to correct this deficiency with ex vivo incubation of CD133+ cells using fucosyltransferase VI and GDP-fucose...
May 5, 2018: Molecular Biotechnology
https://www.readbyqxmd.com/read/29728408/collagen-vi-in-healthy-and-diseased-nervous-system
#7
REVIEW
Ilaria Gregorio, Paola Braghetta, Paolo Bonaldo, Matilde Cescon
Collagen VI is a major extracellular matrix protein exerting a number of functions in different tissues, spanning from biomechanical to regulatory signals in the cell survival processes, and playing key roles in maintaining the stemness or determining the differentiation of several types of cells. In the last couple of years, emerging findings on collagen VI have led to increased interest in its role in the nervous system. The role of this protein in the peripheral nervous system was intensely studied and characterized in detail...
May 4, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29728109/a-serological-biomarker-of-versican-degradation-is-associated-with-mortality-following-acute-exacerbations-of-idiopathic-interstitial-pneumonia
#8
Jannie M B Sand, Yoshinori Tanino, Morten A Karsdal, Takefumi Nikaido, Kenichi Misa, Yuki Sato, Ryuichi Togawa, Xintao Wang, Diana J Leeming, Mitsuru Munakata
BACKGROUND: Idiopathic interstitial pneumonia (IIP) is characterized by an increased rate of extracellular matrix (ECM) remodeling resulting in fibrosis. Acute exacerbations of IIP represent periods of increased disease activity, thus we hypothesized that ECM remodeling was altered during acute exacerbations and investigated this by serological neo-epitope biomarkers. METHODS: Patients who were sequentially admitted to the hospital with acute exacerbations of IIP were retrospectively analyzed for ECM remodeling at time of exacerbation (AE-IIP) and at clinical stability (S-IIP)...
May 4, 2018: Respiratory Research
https://www.readbyqxmd.com/read/29726019/speed-modulation-of-the-heartware-hvad-to-assess-in-vitro-hemocompatibility-of-pulsatile-and-continuous-flow-regimes-in-a-rotary-blood-pump
#9
Jarod T Horobin, Michael J Simmonds, Deepika Nandakumar, Shaun D Gregory, Geoff Tansley, Jo P Pauls, Angela Girnghuber, Nicoletta Balletti, John F Fraser
Although rotary blood pumps (RBPs) sustain life, blood exposure to continuous supra-physiological shear stress induces adverse effects (e.g., thromboembolism); thus, pulsatile flow in RBPs represents a potential solution. The present study introduced pulsatile flow to the HeartWare HVAD using a custom-built controller and compared hemocompatibility biomarkers (i.e., platelet aggregation, concentrations for ADAMTS13, von Willebrand factor (vWf), and free-hemoglobin in plasma (pfHb), red blood cell (RBC) deformability, and RBC-nitric oxide synthase (NOS) activity) between continuous and pulsatile flow in a blood circulation loop over 5 h...
May 3, 2018: Artificial Organs
https://www.readbyqxmd.com/read/29695020/significant-hypo-responsiveness-to-gpvi-and-clec-2-agonists-in-pre-term-and-full-term-neonatal-platelets-and-following-immune-thrombocytopenia
#10
Alexander T Hardy, Verónica Palma-Barqueros, Stephanie K Watson, Jean-Daniel Malcor, Johannes A Eble, Elizabeth E Gardiner, José E Blanco, Rafael Guijarro-Campillo, Juan L Delgado, María L Lozano, Raúl Teruel-Montoya, Vicente Vicente, Steve P Watson, José Rivera, Francisca Ferrer-Marín
Neonatal platelets are hypo-reactive to the tyrosine kinase-linked receptor agonist collagen. Here, we have investigated whether the hypo-responsiveness is related to altered levels of glycoprotein VI (GPVI) and integrin α2β1, or to defects in downstream signalling events by comparison to platelet activation by C-type lectin-like receptor 2 (CLEC-2). GPVI and CLEC-2 activate a Src- and Syk-dependent signalling pathway upstream of phospholipase C (PLC) γ2. Phosphorylation of a conserved YxxL sequence known as a (hemi) immunotyrosine-based-activation-motif (ITAM) in both receptors is critical for Syk activation...
April 25, 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29693792/comparative-proteomic-analyses-of-human-adipose-extracellular-matrices-decellularized-using-alternative-procedures
#11
Caasy Thomas-Porch, Jie Li, Fabiana Zanata, Elizabeth C Martin, Nicholas Pashos, Kaylynn Genemaras, J Nicholas Poche, Nicholas P Totaro, Melyssa R Bratton, Dina Gaupp, Trivia Frazier, Xiying Wu, Lydia Masako Ferreira, Weidong Tian, Guangdi Wang, Bruce A Bunnell, Lauren Flynn, Daniel Hayes, Jeffrey M Gimble
Decellularized human adipose tissue has potential clinical utility as a processed biological scaffold for soft tissue cosmesis, grafting and reconstruction. Adipose tissue decellularization has been accomplished using enzymatic-, detergent-, and/or solvent-based methods. To examine the hypothesis that distinct decellularization processes may yield scaffolds with differing compositions, the current study employed mass spectrometry to compare the proteomes of human adipose-derived matrices generated through three independent methods combining enzymatic-, detergent-, and/or solvent-based steps...
April 25, 2018: Journal of Biomedical Materials Research. Part A
https://www.readbyqxmd.com/read/29673351/fk506-binding-protein-10-fkbp10-regulates-lung-fibroblast-migration-via-collagen-vi-synthesis
#12
Larissa Knüppel, Katharina Heinzelmann, Michael Lindner, Rudolf Hatz, Jürgen Behr, Oliver Eickelberg, Claudia A Staab-Weijnitz
BACKGROUND: In idiopathic pulmonary fibrosis (IPF), fibroblasts gain a more migratory phenotype and excessively secrete extracellular matrix (ECM), ultimately leading to alveolar scarring and progressive dyspnea. Here, we analyzed the effects of deficiency of FK506-binding protein 10 (FKBP10), a potential IPF drug target, on primary human lung fibroblast (phLF) adhesion and migration. METHODS: Using siRNA, FKBP10 expression was inhibited in phLF in absence or presence of 2ng/ml transforming growth factor-β1 (TGF-β1) and 0...
April 19, 2018: Respiratory Research
https://www.readbyqxmd.com/read/29659864/type-vi-collagen-%C3%AE-1-chain-polypeptide-in-non-triple-helical-form-is-an-alternative-gene-product-of-col6a1
#13
Takamichi Sato, Ryo Takano, Kazuhiro Tokunaka, Kan Saiga, Arihiro Tomura, Hidemitsu Sugihara, Toshihiko Hayashi, Yasutada Imamura, Makoto Morita
Expression of type IV collagen α1 chain in non-triple helical form, NTH α1(IV), is observed in cultured human cells, human placenta, and rabbit tissues. Biological functions of NTH α1(IV) are most likely to be distinct from type IV collagen, since their biochemical characteristics are quite different. To explore the biological functions of NTH α1(IV), we prepared some anti-NTH α1(IV) antibodies. In the course of characterization of these antibodies, one antibody, #141, bound to a polypeptide of 140 kDa in size in addition to NTH α1(IV)...
April 5, 2018: Journal of Biochemistry
https://www.readbyqxmd.com/read/29643059/higher-collagen-vi-formation-is-associated-with-all-cause-mortality-in-patients-with-type-2-diabetes-and-microalbuminuria
#14
Daniel G K Rasmussen, Tine W Hansen, Bernt J von Scholten, Signe H Nielsen, Henrik Reinhard, Hans-Henrik Parving, Martin Tepel, Morten A Karsdal, Peter K Jacobsen, Federica Genovese, Peter Rossing
OBJECTIVE: Type 2 diabetes is a common risk factor for the development of chronic kidney disease (CKD). Enhanced de novo collagen type VI (COL VI) formation has been associated with renal fibrosis and CKD. We investigated the hypothesis that PRO-C6, a product specifically generated during COL VI formation, is prognostic for adverse outcomes in patients with type 2 diabetes and microalbuminuria. RESEARCH DESIGN AND METHODS: In a prospective, observational study, we measured PRO-C6 in serum ( S -PRO-C6) and urine (U-PRO-C6) of 198 patients with type 2 diabetes and microalbuminuria without symptoms of coronary artery disease...
April 11, 2018: Diabetes Care
https://www.readbyqxmd.com/read/29624713/clinical-and-imaging-hallmarks-of-the-myh7-related-myopathy-with-severe-axial-involvement
#15
Ivana Dabaj, Robert Y Carlier, David Gómez-Andrés, Osório Abath Neto, Enrico Bertini, Adele D'Amico, Fabiana Fattori, Yann Péréon, Claudia Castiglioni, Eliana Rodillo, Michela Catteruccia, Júlio Brandão Guimarães, Acary Souza Bulle Oliveira, Umbertina Conti Reed, Lilia Mesrob, Doris Lechner, Anne Boland, Jean-François Deleuze, Edoardo Malfatti, Carsten Bonnemann, Jocelyn Laporte, Norma Romero, Adrien Felter, Susana Quijano-Roy, Cristiane Araújo Martins Moreno, Edmar Zanoteli
INTRODUCTION: MYH7 gene mutations are related to a heterogeneous group of skeletal and cardiac myopathies. METHODS: We evaluated clinical and muscle magnetic resonance imaging changes in patients with mutations in the rod domain of MYH7 including one with mosaicism and three with novel missense mutations. RESULTS: Patients presented in childhood with a distal and axial phenotype. Biopsy findings were variable. Half of the cases displaying some type of core pathology, including minicores and eccentric cores...
April 6, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29620224/role-of-col6a3-in-colorectal-cancer
#16
Wei Liu, Li Li, Hua Ye, Huan Tao, Huaqin He
Public transcriptome databases provide a valuable resource for genome‑wide co‑expression network analysis and investigation of the molecular mechanisms that underlie pathogenesis. To discover genes that may affect patient survival, a large‑scale analysis of human colorectal cancer (CRC) datasets that were retrieved from the NCBI Gene Expression Omnibus was performed. A gene co‑expression network was constructed using weighted gene co‑expression network analysis (WGCNA). A total of 18 co‑expressed gene modules were identified, of which two genes corresponded to cell migration and the cell cycle, two genes were involved in immune responses, two genes corresponded to mitochondrial function, and one gene corresponded to RNA splicing...
March 23, 2018: Oncology Reports
https://www.readbyqxmd.com/read/29604837/interaction-of-von-willebrand-factor-domains-with-collagen-investigated-by-single-molecule-force-spectroscopy
#17
Sandra Posch, Tobias Obser, Gesa König, Reinhard Schneppenheim, Robert Tampé, Peter Hinterdorfer
von Willebrand factor (VWF) is a huge multimeric protein that plays a key role in primary hemostasis. Sites for collagen binding, an initial event of hemostasis, are located in the VWF-domains A1 and A3. In this study, we investigated single molecule interactions between collagen surfaces and wild type VWF A1A2A3 domain constructs, as well as clinically relevant VWF A3 domain point mutations, such as p.Ser1731Thr, p.Gln1734His, and p.His1786Arg. For this, we utilized atomic force microscopy based single molecular force spectroscopy...
March 28, 2018: Journal of Chemical Physics
https://www.readbyqxmd.com/read/29588876/ectopic-telomerase-expression-fails-to-maintain-chondrogenic-capacity-in-three-dimensional-cultures-of-clinically-relevant-cell-types
#18
Tina P Dale, Nicholas R Forsyth
The poor healing capacity of cartilage and lack of effective treatment for associated disease and trauma makes it a strong candidate for a regenerative medicine approach. Potential therapies tested to date, although effective, have met with a number of intrinsic difficulties possibly related to limited autologous chondrocyte cell yield and quality of cartilage produced. A potential mechanism to bypass limited cell yields and improve quality of differentiation is to immortalize relevant cell types through the ectopic expression of telomerase...
2018: BioResearch Open Access
https://www.readbyqxmd.com/read/29552635/isolation-and-identification-of-proteins-secreted-by-cells-cultured-within-synthetic-hydrogel-based-matrices
#19
Lisa A Sawicki, Leila H Choe, Katherine L Wiley, Kelvin H Lee, April M Kloxin
Cells interact with and remodel their microenvironment, degrading large extracellular matrix (ECM) proteins (e.g., fibronectin, collagens) and secreting new ECM proteins and small soluble factors (e.g., growth factors, cytokines). Synthetic mimics of the ECM have been developed as controlled cell culture platforms for use in both fundamental and applied studies. However, how cells broadly remodel these initially well-defined matrices remains poorly understood and difficult to probe. In this work, we have established methods for widely examining both large and small proteins that are secreted by cells within synthetic matrices...
March 12, 2018: ACS Biomaterials Science & Engineering
https://www.readbyqxmd.com/read/29521546/collagen-type-vi-interaction-improves-human-islet-survival-in-immunoisolating-microcapsules-for-treatment-of-diabetes
#20
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 4, 2018: Islets
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