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https://www.readbyqxmd.com/read/27893734/an-ipsc-derived-vascular-model-of-marfan-syndrome-identifies-key-mediators-of-smooth-muscle-cell-death
#1
Alessandra Granata, Felipe Serrano, William George Bernard, Madeline McNamara, Lucinda Low, Priya Sastry, Sanjay Sinha
Marfan syndrome (MFS) is a heritable connective tissue disorder caused by mutations in FBN1, which encodes the extracellular matrix protein fibrillin-1. To investigate the pathogenesis of aortic aneurysms in MFS, we generated a vascular model derived from human induced pluripotent stem cells (MFS-hiPSCs). Our MFS-hiPSC-derived smooth muscle cells (SMCs) recapitulated the pathology seen in Marfan aortas, including defects in fibrillin-1 accumulation, extracellular matrix degradation, transforming growth factor-β (TGF-β) signaling, contraction and apoptosis; abnormalities were corrected by CRISPR-based editing of the FBN1 mutation...
November 28, 2016: Nature Genetics
https://www.readbyqxmd.com/read/27893044/failure-of-the-porcine-ascending-aorta-multidirectional-experiments-and-a-unifying-microstructural-model
#2
Colleen M Witzenburg, Rohit Y Dhume, Sachin B Shah, Christopher E Korenczuk, Hallie P Wagner, Patrick W Alford, Victor H Barocas
The ascending thoracic aorta is poorly understood mechanically, especially its risk of dissection. To make better predictions of dissection risk, more information about the multi-dimensional failure behavior of the tissue is needed, and this information must be incorporated into an appropriate theoretical / computational model. Towards the creation of such a model, uniaxial, equibiaxial, peel, and shear lap tests were performed on healthy porcine ascending aorta samples. Uniaxial and equibiaxial tests showed anisotropy with greater stiffness and strength in the circumferential direction...
November 18, 2016: Journal of Biomechanical Engineering
https://www.readbyqxmd.com/read/27892729/reduction-and-fragmentation-of-elastic-fibers-in-the-skin-of-obese-mice-is-associated-with-altered-mrna-expression-levels-of-fibrillin-1-and-neprilysin
#3
Hiroko Makihara, Moeko Hidaka, Yui Sakai, Yoshiko Horie, Hideaki Mitsui, Kenichi Ohashi, Yoshio Goshima, Tomoko Akase
AIM OF THE STUDY: Our previous researches suggested that obesity induces structural fragility in the skin. Elastic fibers impart strength and elasticity. In this study, we determined whether elastic fibers decrease in the skin of obese mice. MATERIALS AND METHODS: To confirm alterations in elastic fiber content due to obesity, we used spontaneously obese model mice (TSOD) and control mice (TSNO). Furthermore, to evaluate the elastin structure and gene expression dependent on the severity of obesity, an obesity-enhanced mouse model was developed by feeding a high fat diet to TSOD (TSOD-HF)...
November 28, 2016: Connective Tissue Research
https://www.readbyqxmd.com/read/27879251/altered-smooth-muscle-cell-force-generation-as-a-driver-of-thoracic-aortic-aneurysms-and-dissections
#4
REVIEW
Dianna M Milewicz, Kathleen M Trybus, Dongchuan Guo, H Lee Sweeney, Ellen Regalado, Kristine Kamm, James T Stull
The importance of maintaining contractile function in aortic smooth muscle cells (SMCs) is evident by the fact that heterozygous mutations in the major structural proteins or kinases controlling contraction lead to the formation of aneurysms of the ascending thoracic aorta that predispose to life-threatening aortic dissections. Force generation by SMC requires ATP-dependent cyclic interactions between filaments composed of SMC-specific isoforms of α-actin (encoded by ACTA2) and myosin heavy chain (MYH11). ACTA2 and MYH11 mutations shown to disrupt this cyclic interaction predispose to thoracic aortic disease...
November 22, 2016: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/27860350/enhanced-sealing-by-hydrophobic-modification-of-alaska-pollock-derived-gelatin-based-surgical-sealants-for-the-treatment-of-pulmonary-air-leaks
#5
Ryo Mizuta, Tetsushi Taguchi
Pulmonary air leaks are medical complications of thoracic surgery for which fibrin sealant is the main treatment. In this study, innovative sealants based on hydrophobically modified Alaska pollock-derived gelatin (hm-ApGltn) and a poly(ethylene)glycol-based 4-armed cross-linker (4S-PEG) have been developed and their burst strengths have been evaluated using fresh rat lung. The developed sealants show higher lung burst strength compared with the nonmodified original ApGltn (Org-ApGltn)-based sealant and a commercial fibrin sealant...
November 15, 2016: Macromolecular Bioscience
https://www.readbyqxmd.com/read/27832158/arnica-montana-stimulates-extracellular-matrix-gene-expression-in-a-macrophage-cell-line-differentiated-to-wound-healing-phenotype
#6
Marta Marzotto, Clara Bonafini, Debora Olioso, Anna Baruzzi, Laura Bettinetti, Francesca Di Leva, Elisabetta Galbiati, Paolo Bellavite
Arnica montana (Arnica m.) is used for its purported anti-inflammatory and tissue healing actions after trauma, bruises, or tissue injuries, but its cellular and molecular mechanisms are largely unknown. This work tested Arnica m. effects on gene expression using an in vitro model of macrophages polarized towards a "wound-healing" phenotype. The monocyte-macrophage human THP-1 cell line was cultured and differentiated with phorbol-myristate acetate and Interleukin-4, then exposed for 24h to Arnica m. centesimal (c) dilutions 2c, 3c, 5c, 9c, 15c or Control...
2016: PloS One
https://www.readbyqxmd.com/read/27826254/elastin-in-the-liver
#7
REVIEW
Jiří Kanta
A characteristic feature of liver cirrhosis is the accumulation of large amounts of connective tissue with the prevailing content of type I collagen. Elastin is a minor connective tissue component in normal liver but it is actively synthesized by hepatic stellate cells and portal fibroblasts in diseased liver. The accumulation of elastic fibers in later stages of liver fibrosis may contribute to the decreasing reversibility of the disease with advancing time. Elastin is formed by polymerization of tropoelastin monomers...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27824871/aortic-and-cardiac-structure-and-function-using-high-resolution-echocardiography-and-optical-coherence-tomography-in-a-mouse-model-of-marfan-syndrome
#8
Ling Lee, Jason Z Cui, Michelle Cua, Mitra Esfandiarei, Xiaoye Sheng, Winsey Audrey Chui, Michael Haoying Xu, Marinko V Sarunic, Mirza Faisal Beg, Cornelius van Breemen, George G S Sandor, Glen F Tibbits
Marfan syndrome (MFS) is an autosomal-dominant disorder of connective tissue caused by mutations in the fibrillin-1 (FBN1) gene. Mortality is often due to aortic dissection and rupture. We investigated the structural and functional properties of the heart and aorta in a [Fbn1C1039G/+] MFS mouse using high-resolution ultrasound (echo) and optical coherence tomography (OCT). Echo was performed on 6- and 12-month old wild type (WT) and MFS mice (n = 8). In vivo pulse wave velocity (PWV), aortic root diameter, ejection fraction, stroke volume, left ventricular (LV) wall thickness, LV mass and mitral valve early and atrial velocities (E/A) ratio were measured by high resolution echocardiography...
2016: PloS One
https://www.readbyqxmd.com/read/27812333/fibrillins-in-tendon
#9
REVIEW
Betti Giusti, Guglielmina Pepe
Tendons among connective tissue, mainly collagen, contain also elastic fibers (EF) made of fibrillin 1, fibrillin 2 and elastin that are broadly distributed in tendons and represent 1-2% of the dried mass of the tendon. Only in the last years, studies on structure and function of EF in tendons have been performed. Aim of this review is to revise data on the organization of EF in tendons, in particular fibrillin structure and function, and on the clinical manifestations associated to alterations of EF in tendons...
2016: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/27798118/heat-delays-skin-wound-healing-in-mice
#10
Marco Aurélio Dos Santos-Silva, Eduardo Tavares Lima Trajano, Fernanda Seabra Schanuel, Andréa Monte-Alto-Costa
In vivo studies have shown that the combination of infrared radiation (IR) and visible light (VIS) is responsible for the activation of metaloproteinases, causing matrix degradation and damage to healthy skin. However, the role of heat originating from the VIS spectrum on wound healing remains poorly understood. Our objective was to investigate the macroscopic, microscopic and biochemical effects of heat induced by visible light on cutaneous wound healing in mice. Male mice were anesthetized, subjected to a cutaneous excisional wound and divided into two groups (n = 10/group) exposed to 23℃ or 43℃ in a thermal chamber for 30 min every other day, for 13 days...
October 21, 2016: Experimental Biology and Medicine
https://www.readbyqxmd.com/read/27790800/striae-as-a-predictive-clinical-marker-for-urogenital-dystopia-and-preventive-treatment-proposal
#11
P R Cunha, C B Mattos, A F Salai
Striae are common dermal lesions of unknown etiology. These stretch marks are linear and atrophic plaques due to the continuous and progressive distension of the skin (1,2,3). Their pathogenesis involves reductions and modifications in the configuration of the elastic fibers, collagen, and fibrillin of the dermis (3, 4), structures which are produced by fibroblasts. Striae and dystopic urogenital tissues have inactive fibroblasts causing deficiency in the contractility of the two conditions (4). This article is protected by copyright...
October 28, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27788826/morphologic-changes-in-the-dermis-after-the-single-administration-of-autologous-fibroblastic-cells-a-preliminary-study
#12
B Machaliński, A Brodkiewicz, K Szumilas, D Rogińska, M P Kawa, I Stecewicz, G Trybek, M Marchlewicz, B Wiszniewska
BACKGROUND: Aging is a multifactorial process defined by an accumulation of damage in all tissues and organs, including the skin, throughout the lifespan of an individual. The reduction of both cellular and extracellular matrix components of the dermis during the aging process is followed by the alteration of the morphology of the skin tissue. This study was conducted to assess skin morphology in men before and 3 months after the intradermal injection of autologous fibroblastic cells...
October 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27779234/adamts-10-and-6-differentially-regulate-cell-cell-junctions-and-focal-adhesions
#13
Stuart A Cain, Ewa J Mularczyk, Mukti Singh, Teresa Massam-Wu, Cay M Kielty
ADAMTS10 and ADAMTS6 are homologous metalloproteinases with ill-defined roles. ADAMTS10 mutations cause Weill-Marchesani syndrome (WMS), implicating it in fibrillin microfibril biology since some fibrillin-1 mutations also cause WMS. However little is known about ADAMTS6 function. ADAMTS10 is resistant to furin cleavage, however we show that ADAMTS6 is effectively processed and active. Using siRNA, over-expression and mutagenesis, it was found ADAMTS6 inhibits and ADAMTS10 is required for focal adhesions, epithelial cell-cell junction formation, and microfibril deposition...
October 25, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27751812/induction-of-fibrillin-2-and-periostin-expression-in-osterix-knockdown-mc3t3-e1-cells
#14
So-Jeong Lee, Eun-Hye Lee, Seung-Yoon Park, Jung-Eun Kim
Osteoporosis is the most common age-related bone disease that is characterized by an imbalance between osteoblasts for bone formation and osteoclasts for bone resorption. Anti-catabolic drugs have been developed to inhibit osteoclast activity and to prevent bone loss in osteoporosis. However, because it is difficult to increase bone mass in osteoporotic bone, it would be beneficial to simultaneously enhance osteoblast function and thus form bone. Osterix (Osx) is an essential transcription factor for osteoblast differentiation...
October 14, 2016: Gene
https://www.readbyqxmd.com/read/27704402/the-effect-of-losartan-therapy-on-ventricular-function-in-marfan-patients-with-haploinsufficient-or-dominant-negative-fbn1-mutations
#15
A W den Hartog, R Franken, M P van den Berg, A H Zwinderman, J Timmermans, A J Scholte, V de Waard, A M Spijkerboer, G Pals, B J M Mulder, M Groenink
BACKGROUND: Mild biventricular dysfunction is often present in patients with Marfan syndrome. Losartan has been shown to reduce aortic dilatation in patients with Marfan syndrome. This study assesses the effect of losartan on ventricular volume and function in genetically classified subgroups of asymptomatic Marfan patients without significant valvular regurgitation. METHODS: In this predefined substudy of the COMPARE study, Marfan patients were classified based on the effect of their FBN1 mutation on fibrillin-1 protein, categorised as haploinsufficient or dominant negative...
November 2016: Netherlands Heart Journal
https://www.readbyqxmd.com/read/27677855/independent-multimerization-of-latent-tgf%C3%AE-binding-protein-1-stabilized-by-cross-linking-and-enhanced-by-heparan-sulfate
#16
Helen Troilo, Ruth Steer, Richard F Collins, Cay M Kielty, Clair Baldock
TGFβ plays key roles in fibrosis and cancer progression, and latency is conferred by covalent linkage to latent TGFβ binding proteins (LTBPs). LTBP1 is essential for TGFβ folding, secretion, matrix localization and activation but little is known about its structure due to its inherent size and flexibility. Here we show that LTBP1 adopts an extended conformation with stable matrix-binding N-terminus, extended central array of 11 calcium-binding EGF domains and flexible TGFβ-binding C-terminus. Moreover we demonstrate that LTBP1 forms short filament-like structures independent of other matrix components...
September 28, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27645114/ltbp4-regulates-pdgfr%C3%AE-expression-via-tgf%C3%AE-dependent-modulation-of-nrf2-transcription-factor-function
#17
Ana Tomasovic, Nina Kurrle, Frank Wempe, Silke De-Zolt, Susan Scheibe, Katri Koli, Martin Serchinger, Frank Schnütgen, Duran Sürün, Anja Sterner-Kock, Norbert Weissmann, Harald von Melchner
Latent transforming growth factor beta binding protein 4 (LTBP4) belongs to the fibrillin/LTBP family of proteins and plays an important role as a structural component of extracellular matrix (ECM) and local regulator of TGFβ signaling. We have previously reported that Ltbp4S knock out mice (Ltbp4S-/-) develop centrilobular emphysema reminiscent of late stage COPD, which could be partially rescued by inactivating the antioxidant protein Sestrin 2 (Sesn2). More recent studies showed that Sesn2 knock out mice upregulate Pdgfrβ-controlled alveolar maintenance programs that protect against cigarette smoke induced pulmonary emphysema...
September 16, 2016: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/27634926/fibrillin-2-is-upregulated-in-the-ascending-aorta-of-patients-with-bicuspid-aortic-valve
#18
Carmen Rueda-Martínez, Oscar Lamas, María José Mataró, Juan Robledo-Carmona, Gemma Sánchez-Espín, Inmaculada Moreno-Santos, Fernando Carrasco-Chinchilla, Pastora Gallego, Miguel Such-Martínez, Eduardo de Teresa, Manuel Jiménez-Navarro, Borja Fernández
OBJECTIVES: Bicuspid aortic valve (BAV) is the most prevalent congenital cardiac malformation, frequently associated with aortic dilatation (AD). The molecular mechanisms involved in AD and its aetiological link with BAV formation are poorly understood. Altered fibrillin-1 (FBN1) and metalloprotease-2, -9 (MMP2,9) protein activities have been suggested to be involved in BAV aortopathy. In addition, FBN2 participates in embryonic valve formation, but its possible involvement in BAV-associated AD has never been explored...
September 15, 2016: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/27615407/marfan-syndrome-report-of-a-complex-phenotype-due-to-a-15q21-1-contiguos-gene-deletion-encompassing-fbn1-and-literature-review
#19
Chiara Dordoni, Claudia Ciaccio, Graziano Santoro, Marina Venturini, Ugo Cavallari, Marco Ritelli, Marina Colombi
Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder that primarily involves skeletal, ocular, and cardiovascular systems with large inter- and intra-familial variability in terms of age of onset, severity, and aortic disease. The causal gene, FBN1, encodes for fibrillin 1, a multi-domain glycoprotein essential for many biological functions, including deposition and formation of elastic fibers. Reports describing chromosomal alterations involving FBN1 are rare, but in the last years their number has increased after copy number state analyses, such as multiplex ligation-dependent probe amplification and microarray-based comparative genomic hybridization, were adopted as routine diagnostic tools...
September 12, 2016: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/27606123/seizures-as-an-atypical-feature-of-beal-s-syndrome
#20
Nazreen B K Jaman, Abeer Al-Sayegh
Congenital contractural arachnodactyly, commonly known as Beal's syndrome, is an extremely rare genetic disorder caused by mutations in the fibrillin-2 (FBN2) gene located on chromosome 5q23. It is an autosomal dominant inherited connective tissue disorder characterised by a Marfan-like body habitus, contractures, abnormally shaped ears and kyphoscoliosis. We report a seven-year-old Omani male who presented to the Sultan Qaboos University Hospital, Muscat, Oman, in 2014 with seizures. He was noted to have certain distinctive facial features and musculoskeletal manifestations; he was subsequently diagnosed with Beal's syndrome...
August 2016: Sultan Qaboos University Medical Journal
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