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Collagen VI

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https://www.readbyqxmd.com/read/29139382/-episodes-of-recurrent-pneumothorax-in-a-patient-with-collagen-vi-related-congenital-muscular-dystrophy
#1
Rémi Bellance, Rudy Valentino, Bruno Sanchez, Octavio Labrada-Blanco, Linda Manere, J Andoni Urtizberea, Elisabeth Sarrazin
No abstract text is available yet for this article.
November 2017: Médecine Sciences: M/S
https://www.readbyqxmd.com/read/29132420/substance-p-and-patterned-silk-biomaterial-stimulate-periodontal-ligament-stem-cells-to-form-corneal-stroma-in-a-bioengineered-three-dimensional-model
#2
Jialin Chen, Wei Zhang, Peyman Kelk, Ludvig J Backman, Patrik Danielson
BACKGROUND: We aimed to generate a bioengineered multi-lamellar human corneal stroma tissue in vitro by differentiating periodontal ligament stem cells (PDLSCs) towards keratocytes on an aligned silk membrane. METHODS: Human PDLSCs were isolated and identified. The neuropeptide substance P (SP) was added in keratocyte differentiation medium (KDM) to evaluate its effect on keratocyte differentiation of PDLSCs. PDLSCs were then seeded on patterned silk membrane and cultured with KDM and SP...
November 13, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/29129153/skin-biopsy-for-diagnosis-of-ullrich-congenital-muscular-dystrophy-an-observational-study
#3
Biswaroop Chakrabarty, M C Sharma, Sheffali Gulati, Chitra Sarkar
The gold standard diagnostic test for Ullrich congenital muscular dystrophy (UCMD) is molecular testing for COL6 mutation. The facility for genetic testing is sparingly available and it is usually diagnosed by muscle biopsy. The latter is an invasive procedure requiring expertise and sedation. Skin biopsy has shown promise as a simpler diagnostic modality. Eleven and 7 cases, respectively, of phenotypically suspected Ullrich congenital muscular dystrophy and dystrophinopathy underwent simultaneous skin and muscle biopsies, which were subjected to hematoxylin and eosin (H&E) and immunohistochemistry staining for collagen VI and dystrophin 1, 2, and 3...
December 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/29127443/astragaloside-iv-attenuates-orbital-inflammation-in-graves-orbitopathy-through-suppression-of-autophagy
#4
Hong Li, Yali Zhang, Jie Min, Long Gao, Ren Zhang, Yucheng Yang
INTRODUCTION: Graves' orbitopathy (GO) is an autoimmune inflammatory disorder affecting the orbit around the eye. Astragaloside IV (AS-VI) is the main active ingredient of the Chinese herbal medicine Huangqi (Radix Astragali Mongolici). AS-IV exhibits antioxidant and anti-inflammatory properties, and shows therapeutic potential in a number of ischemic and inflammatory diseases; however, its pharmaceutical activities in GO remain undefined. MATERIALS AND METHODS: In this study, we investigated the effects of AS-IV on interleukin (IL)-1β-induced orbital fibroblast inflammation in vitro and GO orbital inflammation and ocular histopathological changes in vivo, as well as the underlying mechanisms responsible for these effects...
November 10, 2017: Inflammation Research: Official Journal of the European Histamine Research Society ... [et Al.]
https://www.readbyqxmd.com/read/29122677/inducible-knockdown-of-procollagen-i-protects-mice-from-liver-fibrosis-and-leads-to-dysregulated-matrix-genes-and-attenuated-inflammation
#5
Olena Molokanova, Kai Schönig, Shih-Yen Weng, Xiaoyu Wang, Matthias Bros, Mustafa Diken, Svetlana Ohngemach, Morten Karsdal, Dennis Strand, Alexei Nikolaev, Leonid Eshkind, Detlef Schuppan
Organ fibrosis is characterized by a chronic wound-healing response, with excess deposition of extracellular matrix components. Here, collagen type I represents the most abundant scar component and a primary target for antifibrotic therapies. Liver fibrosis can progress to cirrhosis and primary liver cancer, which are the major causes of liver related morbidity and mortality. However, a (pro-)collagen type I specific therapy remains difficult and its therapeutic abrogation may incur unwanted side effects. We therefore designed tetracycline-regulated procollagen alpha1(I) short hairpin (sh)RNA expressing mice that permit a highly efficient inducible knockdown of the procollagen alpha1(I) gene in activated (myo-)fibroblasts, to study the effect of induced procollagen type I deficiency...
November 6, 2017: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29117645/proteomic-profiling-of-tgfbi-null-mouse-corneas-reveals-only-minor-changes-in-matrix-composition-supportive-of-tgfbi-knockdown-as-therapy-against-tgfbi-linked-corneal-dystrophies
#6
Ebbe Toftgaard Poulsen, Kasper Runager, Nadia Sukusu Nielsen, Marie V Lukassen, Karen Thomsen, Paige Snider, Olga Simmons, Henrik Vorum, Simon J Conway, Jan J Enghild
TGFBIp is a constituent of the extracellular matrix in many human tissues including the cornea, where it is one of the most abundant proteins expressed. TGFBIp interacts with Type I, II, IV, VI and XII collagens as well as several members of the integrin family, suggesting it plays an important role in maintaining structural integrity and possibly corneal transparency as well. Significantly, more than 60 point mutations within the TGFBI gene have been reported to result in aberrant TGFBIp folding and aggregation in the cornea, resulting in severe visual impairment and blindness...
November 8, 2017: FEBS Journal
https://www.readbyqxmd.com/read/29114203/collagen-vi-null-mice-as-a-model-for-early-onset-muscle-decline-in-aging
#7
Daniele Capitanio, Manuela Moriggi, Sara De Palma, Dario Bizzotto, Sibilla Molon, Enrica Torretta, Chiara Fania, Paolo Bonaldo, Cecilia Gelfi, Paola Braghetta
Collagen VI is an extracellular matrix (ECM) protein playing a key role in skeletal muscles and whose deficiency leads to connective tissue diseases in humans and in animal models. However, most studies have been focused on skeletal muscle features. We performed an extensive proteomic profiling in two skeletal muscles (diaphragm and gastrocnemius) of wild-type and collagen VI null (Col6a1(-/-)) mice at different ages, from 6- (adult) to 12- (aged) month-old to 24 (old) month-old. While in wild-type animals the number of proteins and the level of modification occurring during aging were comparable in the two analyzed muscles, Col6a1(-/-) mice displayed a number of muscle-type specific variations...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29107969/assessing-single-stranded-oligonucleotide-drug-induced-effects-in-vitro-reveals-key-risk-factors-for-thrombocytopenia
#8
Sabine Sewing, Adrian B Roth, Michael Winter, Andreas Dieckmann, Cristina Bertinetti-Lapatki, Yann Tessier, Claudia McGinnis, Sylwia Huber, Erich Koller, Corinne Ploix, John C Reed, Thomas Singer, Andreas Rothfuss
Single-stranded oligonucleotides (ON) comprise a promising therapeutic platform that enables selective modulation of currently undruggable targets. The development of novel ON drug candidates has demonstrated excellent efficacy, but in certain cases also some safety liabilities were reported. Among them are events of thrombocytopenia, which have recently been evident in late stage trials with ON drugs. The underlying mechanisms are poorly understood and the risk for ON candidates causing such events cannot be sufficiently assessed pre-clinically...
2017: PloS One
https://www.readbyqxmd.com/read/29052937/targeting-of-c-type-lectin-like-receptor-2-or-p2y12-for-the-prevention-of-platelet-activation-by-immunotherapeutic-cpg-oligodeoxynucleotides-comment
#9
U Flierl, T L Nero, B Lim, R K Andrews, M W Parker, E E Gardiner, K Peter
We read with great interest the article by Delierneux et al. [1] in the Journal of Thrombosis and Haemostasis. The authors elegantly describe a mechanism of platelet activation by CpG oligodeoxynucleotides (ODNs) through CLEC-2 mediated binding and uptake. This is the second platelet activation mechanism to be attributed to CpG ODNs, the first being the one described by our group involving platelet-specific collagen receptor glycoprotein VI (GPVI) [2]. CpG ODNs are short single-stranded DNA molecules resembling bacterial DNA and they were developed as potential drug candidates, largely due to their immunostimulatory properties via activation of toll-like receptor 9 (TLR9) [3]...
October 20, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29051516/stromal-striae-a-new-insight-into-corneal-physiology-and-mechanics
#10
Kate Grieve, Djida Ghoubay, Cristina Georgeon, Gael Latour, Amir Nahas, Karsten Plamann, Caroline Crotti, Romain Bocheux, Marie Borderie, Thu-Mai Nguyen, Felipe Andreiuolo, Marie-Claire Schanne-Klein, Vincent Borderie
We uncover the significance of a previously unappreciated structural feature in corneal stroma, important to its biomechanics. Vogt striae are a known clinical indicator of keratoconus, and consist of dark, vertical lines crossing the corneal depth. However we detected stromal striae in most corneas, not only keratoconus. We observed striae with multiple imaging modalities in 82% of 118 human corneas, with pathology-specific differences. Striae generally depart from anchor points at Descemet's membrane in the posterior stroma obliquely in a V-shape, whereas in keratoconus, striae depart vertically from posterior toward anterior stroma...
October 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29044725/serum-sclerostin-levels-in-adults-with-osteogenesis-imperfecta-comparison-with-normal-individuals-and-response-to-teriparatide-therapy
#11
Lindsey Nicol, Ying Wang, Rosamund Smith, John Sloan, Sandesh C S Nagamani, Jay Shapiro, Brendan Lee, Eric Orwoll
Sclerostin (SOST), a glycoprotein primarily derived from osteocytes, is an important regulator of bone remodeling. Osteogenesis imperfecta ([1]) is a heritable disorder of bone characterized by low bone mass, bone fragility, recurrent fractures, and bone deformities. Altered SOST-mediated signaling may have a role in pathogenesis of type I collagen-related OI; however this has not been evaluated in humans. We measured serum SOST levels in adults with OI who were enrolled in a randomized, placebo-controlled clinical trial that evaluated the effects of osteoanabolic therapy with teriparatide...
October 17, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/29024862/4-phenylbutyrate-and-valproate-treatment-attenuates-the-progression-of-atherosclerosis-and-stabilizes-existing-plaques
#12
Aric Huang, Tayler L Young, Vi T Dang, Yuanyuan Shi, Cameron S McAlpine, Geoff H Werstuck
BACKGROUND AND AIMS: Recent evidence suggests that endoplasmic reticulum (ER) stress signaling through glycogen synthase kinase (GSK)-3α/β is involved in the activation of pro-atherosclerotic processes. In this study, we examined the effects of small molecules that interfere with ER stress-GSK3α/β signaling on the progression and regression of atherosclerosis in a mouse model. METHODS: To examine atherosclerotic progression, low-density lipoprotein receptor deficient (Ldlr(-/-)) mice were placed on a high-fat diet (HFD) and treated with the chemical chaperone, 4-phenylbutyrate (4PBA, 3...
November 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28989568/expression-of-matrix-metalloproteinase-11-is-increased-under-conditions-of-insulin-resistance
#13
Biagio Arcidiacono, Eusebio Chiefari, Anna Elisa Laria, Sebastiano Messineo, Francesco Luciano Bilotta, Domenico Britti, Daniela Patrizia Foti, Anna Foryst-Ludwig, Ulrich Kintscher, Antonio Brunetti
AIM: To investigate matrix metalloproteinase-11 (MMP-11) expression in adipose tissue dysfunction, using in vitro and in vivo models of insulin resistance. METHODS: Culture of mouse 3T3-L1 preadipocytes were induced to differentiation into mature 3T3-L1 adipocytes. Cellular insulin resistance was induced by treating differentiated cultured adipocytes with hypoxia and/or tumor necrosis factor (TNF)-α, and transcriptional changes were analyzed in each condition thereafter...
September 15, 2017: World Journal of Diabetes
https://www.readbyqxmd.com/read/28984114/a-novel-de-novo-col6a1-mutation-emphasizes-the-role-of-intron-14-donor-splice-site-defects-as-a-cause-of-moderate-progressive-form-of-colvi-myopathy-a-case-report-and-review-of-the-genotype-phenotype-correlation
#14
Agnieszka A Koppolu, Agnieszka Madej-Pilarczyk, Małgorzata Rydzanicz, Joanna Kosińska, Piotr Gasperowicz, Jolanta Dorszewska, Wojciech Kozubski, Barbara Steinborn, Andrzej M Kochański, Rafał Płoski
Collagen VI-related myopathy is a group of disorders affecting skeletal muscles and connective tissue. The most common symptoms are muscle weakness and joint deformities which limit the movement and progress over time. Several forms of collagen VI-related myopathies have been described: Bethlem myopathy, an intermediate form and Ullrich congenital muscular dystrophy, which is the most severe. Here we report a novel de novo c.1056+3A>C substitution in intron 14 of the COL6A1 gene encoding alpha-chains of collagen VI in a 13-year-old girl suffering from collagen VI (ColVI) myopathy...
2017: Folia Neuropathologica
https://www.readbyqxmd.com/read/28981200/soluble-fibrin-causes-an-acquired-platelet-glycoprotein-vi-signaling-defect-implications-for-coagulopathy
#15
M Y Lee, C C Verni, B A Herbig, S L Diamond
Essentials Collagen and thrombin when used simultaneously generate highly activated platelets. The effect of thrombin stimulation on subsequent glycoprotein VI (GPVI) function was observed. Soluble fibrin, but not protease activated receptor (PAR) activation, prevented GPVI activation. Circulating soluble fibrin in coagulopathic blood may cause an acquired GPVI signaling defect. SUMMARY: Background In coagulopathic blood, circulating thrombin may drive platelet dysfunction. Methods/Results Using calcium dye-loaded platelets, the effect of thrombin exposure and soluble fibrin generation on subsequent platelet GPVI function was investigated...
October 5, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28955745/bioengineered-bruch-s-like-extracellular-matrix-promotes-retinal-pigment-epithelial-differentiation
#16
Samuel McLenachan, Erwei Hao, Dan Zhang, Ling Zhang, Michael Edel, Fred Chen
In the eye, the retinal pigment epithelium (RPE) adheres to a complex protein matrix known as Bruch's membrane (BrM). The aim of this study was to provide enriched conditions for RPE cell culture through the production of a BrM-like matrix. Our hypothesis was that a human RPE cell line would deposit an extracellular matrix (ECM) resembling BrM. The composition and structure of ECM deposited by ARPE19 cells (ARPE19-ECM) was characterized. To produce ARPE19-ECM, ARPE19 cells were cultured in the presence dextran sulphate...
July 2017: Biochemistry and Biophysics Reports
https://www.readbyqxmd.com/read/28950757/better-platelet-function-less-fibrinolysis-and-less-hemolysis-in-re-transfused-residual-pump-blood-with-the-ringer-s-chase-technique-a-randomized-pilot-study
#17
Anki Olsson, Joakim Alfredsson, Sofia Ramström, Rolf Svedjeholm, Dermot Kenny, Eric Håkansson, Johan Sanmartin Berglund, Sören Berg
INTRODUCTION: Residual pump blood from the cardiopulmonary bypass (CPB) circuit is often collected into an infusion bag (IB) and re-transfused. An alternative is to chase the residual blood into the circulation through the arterial cannula with Ringer's acetate. Our aim was to assess possible differences in hemostatic blood quality between these two techniques. METHODS: Forty adult patients undergoing elective coronary artery bypass graft surgery with CPB were randomized to receive the residual pump blood by either an IB or through the Ringer's chase (RC) technique...
September 1, 2017: Perfusion
https://www.readbyqxmd.com/read/28947593/mir155-deficiency-aggravates-high-fat-diet-induced-adipose-tissue-fibrosis-in-male-mice
#18
Kandy T Velázquez, Reilly T Enos, Meredith S Carson, Taryn L Cranford, Jackie E Bader, Alexander T Sougiannis, Cara Pritchett, Daping Fan, James A Carson, E Angela Murphy
Noncoding RNAs are emerging as regulators of inflammatory and metabolic processes. There is evidence to suggest that miRNA155 (miR155) may be linked to inflammation and processes associated with adipogenesis. We examined the impact of global miRNA-155 deletion (miR155(-/-)) on the development of high-fat diet (HFD)-induced obesity. We hypothesized that loss of miR155 would decrease adipose tissue inflammation and improve the metabolic profile following HFD feedings. Beginning at 4-5 weeks of age, male miR155(-/-) and wild-type (WT) mice (n = 13-14) on a C57BL/6 background were fed either a HFD or low-fat diet for 20 weeks...
September 2017: Physiological Reports
https://www.readbyqxmd.com/read/28944903/gpvi%C3%A2-fc%C3%A2-peg-improves-cerebral-infarct-volume-and-cerebral-thrombosis-in-mouse-model-with-cerebral-thrombosis
#19
Yimae Wufuer, Xuefeng Shan, Magaoweiya Sailike, Kamile Adilaimu, Songfeng Ma, Huguo Wang
Cerebral thrombosis is one of the most common causes of cerebral infarction, and anticoagulation therapy is a routine treatment in patients with hemorrhagic cerebral venous thrombosis. The hemostatic function of platelets is important for the anticoagulation therapy of thrombosis. Glycoprotein VI (GPVI) is reported as the major signaling receptor for collagen and is exclusively expressed on platelets and megakaryocytes, initiating platelet recruitment at sites of vascular injury and demonstrating numerous beneficial effects for patients with cerebral thrombosis...
November 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28936799/proteomic-features-of-colorectal-cancer-identify-tumor-subtypes-independent-of-oncogenic-mutations-and-independently-predict-relapse-free-survival
#20
Callisia N Clarke, Michael S Lee, Wei Wei, Ganiraju Manyam, Zhi-Qin Jiang, Yiling Lu, Jeffrey Morris, Bradley Broom, David Menter, Eduardo Vilar-Sanchez, Kanwal Raghav, Cathy Eng, George J Chang, Iris Simon, Rene Bernards, Michael Overman, Gordon B Mills, Dipen Maru, Scott Kopetz
BACKGROUND: The directed study of the functional proteome in colorectal cancer (CRC) has identified critical protein markers and signaling pathways; however, the prognostic relevance of many of these proteins remains unclear. METHODS: We determined the prognostic implications of the functional proteome in 263 CRC tumor samples from patients treated at MD Anderson Cancer Center (MDACC) and 462 patients from The Cancer Genome Atlas (TCGA) to identify patterns of protein expression that drive tumorigenesis...
December 2017: Annals of Surgical Oncology
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