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https://www.readbyqxmd.com/read/29933018/surf1-mutations-in-chinese-patients-with-leigh-syndrome-novel-mutations-mutation-spectrum-and-the-functional-consequences
#1
Yuanyuan Li, Shumeng Wen, Dongxiao Li, Jie Xie, Xiujuan Wei, Xiyuan Li, Yi Liu, Hezhi Fang, Yanling Yang, Jianxin Lyu
SURF1 is an assembly factor of mitochondrial complex IV, and its mutations are the primary cause of Leigh syndrome in infants. To date, over 100 SURF1 mutations have been reported worldwide, but the spectrum of the SURF1 mutations in China remains unclear. Here, using next-generation sequencing targeting mitochondrial protein-coding sequences, we sequenced 178 patients suspected to have mitochondrial diseases. Fifteen SURF1 mutations were identified in 12 Leigh syndrome patients, of which three, c.465_466delAA, c...
June 19, 2018: Gene
https://www.readbyqxmd.com/read/29932110/dpie-2-1-2-diphenyl-1h-indol-3-yl-ethanamine-augments-pro-inflammatory-cytokine-production-in-il-1%C3%AE-stimulated-primary-human-oral-cells
#2
Sun-Hee Ahn, Jin-Kyung Lee, Nam Doo Kim, Seok-Ho Kim, Sunwoo Lee, Seunggon Jung, Kee-Oh Chay, Tae-Hoon Lee
Interleukin-1β (IL-1β) is a prominent pro-inflammatory cytokine that is implicated in a variety of autoimmune diseases and plays an important role in host defense against infections. IL-1β activity increases with its increasing binding capacity to IL-1 receptors (IL-1Rs). Thus, numerous studies have targeted the discovery of molecules modulating the interactions between IL-1β and IL-1R1. We have conducted an IL-1R1 structure-based virtual screening to identify small molecules that could alter IL-1β activity, using in silico computational analysis...
June 22, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29931427/premacular-membranes-in-tissue-culture
#3
Denise Vogt, Franziska Vielmuth, Christian Wertheimer, Felix Hagenau, Stefanie R Guenther, Armin Wolf, Volker Spindler, Siegfried G Priglinger, Ricarda G Schumann
PURPOSE: To investigate integrity and characteristics of human premacular membranes (PMM) with and without standard tissue culturing using mechanical traction. METHODS: Premacular membranes were harvested from 32 eyes of 32 patients with idiopathic macular pucker during standard vitrectomy. By flat-mount preparation with phase contrast and interference microscopy, specimens were prepared for time-lapse microscopy, immunocytochemistry, and transmission electron microscopy...
June 21, 2018: Graefe's Archive for Clinical and Experimental Ophthalmology
https://www.readbyqxmd.com/read/29930532/idiopathic-hypersomnia-patients-revealed-longer-circadian-period-length-in-peripheral-skin-fibroblasts
#4
Linus Materna, Hartmut Halfter, Anna Heidbreder, Matthias Boentert, Julian Lippert, Raphael Koch, Peter Young
The vast majority of living organisms have evolved a circadian rhythm of roughly 24 h in adaptation to ever-changing environmental conditions, such as the cycle of light and darkness. In some sleep disorders like idiopathic hypersomnia (IH) this adaptation is defective. As the etiology of this disease is largely unknown, we examined the in vitro circadian period length of patients suffering from IH. The patients were diagnosed according to the ICSD3-criteria by clinical history, polysomnography (PSG), and multiple sleep latency testing (MSLT)...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29925962/expression-of-human-mutant-cyclin-dependent-kinase-4-cyclin-d-and-telomerase-extends-the-life-span-but-does-not-immortalize-fibroblasts-derived-from-loggerhead-sea-turtle-caretta-caretta
#5
Tomokazu Fukuda, Takahiro Eitsuka, Kenichiro Donai, Masanori Kurita, Tomomi Saito, Hitoshi Okamoto, Kodzue Kinoshita, Masafumi Katayama, Hiroshi Nitto, Takafumi Uchida, Manabu Onuma, Hideko Sone, Miho Inoue-Murayama, Tohru Kiyono
Conservation of the genetic resources of endangered animals is crucial for future generations. The loggerhead sea turtle (Caretta caretta) is a critically endangered species, because of human hunting, hybridisation with other sea turtle species, and infectious diseases. In the present study, we established primary fibroblast cell lines from the loggerhead sea turtle, and showed its species specific chromosome number is 2n = 56, which is identical to that of the hawksbill and olive ridley sea turtles. We first showed that intensive hybridization among multiple sea turtle species caused due to the identical chromosome number, which allows existence of stable hybridization among the multiple sea turtle species...
June 20, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29921376/amino-acid-and-hyaluronic-acid-mixtures-differentially-regulate-extra-cellular-matrix-genes-in-cultured-human-fibroblasts
#6
B De Servi, A Orlandini, E Caviola, M Meloni
The aim of this screening study was to evaluate the efficacy of different proprietary mixtures of amino acid and hyaluronic acid (HA) in stimulating the production of extracellular matrix (ECM) components, particularly the neo-synthesis of elastin, and in promoting a more efficient deposition of elastic fibres (elastogenesis), while at the same time maintaining the stimulation of collagen. The study has allowed identification of the optimal ratios between the amino acids (AA) for the production of collagen and elastin...
May 2018: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/29915722/new-therapies-and-immunological-findings-in-cutaneous-t-cell-lymphoma
#7
REVIEW
Kazuyasu Fujii
Primary cutaneous lymphomas comprise a group of lymphatic malignancies that occur primarily in the skin. They represent the second most common form of extranodal non-Hodgkin's lymphoma and are characterized by heterogeneous clinical, histological, immunological, and molecular features. The most common type is mycosis fungoides and its leukemic variant, Sézary syndrome. Both diseases are considered T-helper cell type 2 (Th2) diseases. Not only the tumor cells but also the tumor microenvironment can promote Th2 differentiation, which is beneficial for the tumor cells because a Th1 environment enhances antitumor immune responses...
2018: Frontiers in Oncology
https://www.readbyqxmd.com/read/29915390/gallic-acid-improves-cardiac-dysfunction-and-fibrosis-in-pressure-overload-induced-heart-failure
#8
Li Jin, Simei Sun, Yuhee Ryu, Zhe Hao Piao, Bin Liu, Sin Young Choi, Gwi Ran Kim, Hyung-Seok Kim, Hae Jin Kee, Myung Ho Jeong
Gallic acid is a trihydroxybenzoic acid found in tea leaves and some plants. Here, we report the effect of gallic acid on cardiac dysfunction and fibrosis in a mouse model of pressure overload-induced heart failure and in primary rat cardiac fibroblasts, and compare the effects of gallic acid with those of drugs used in clinics. Gallic acid reduces cardiac hypertrophy, dysfunction, and fibrosis induced by transverse aortic constriction (TAC) stimuli in vivo and transforming growth factor β1 (TGF-β1) in vitro...
June 18, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29914569/clonal-dynamics-studied-in-cultured-induced-pluripotent-stem-cells-reveal-major-growth-imbalances-within-a-few-weeks
#9
David Brenière-Letuffe, Aya Domke-Shibamiya, Arne Hansen, Thomas Eschenhagen, Boris Fehse, Kristoffer Riecken, Justus Stenzig
BACKGROUND: Human induced pluripotent stem (iPS) cells have revolutionised research and spark hopes for future tissue replacement therapies. To obtain high cell numbers, iPS cells can be expanded indefinitely. However, as long-term expansion can compromise cell integrity and quality, we set out to assess potential reduction of clonal diversity by inherent growth imbalances. METHODS: Using red, green, blue marking as a lentiviral multi-colour clonal cell tracking technology, we marked three different iPS cell lines as well as three other cell lines, assigning a unique fluorescent colour to each cell at one point in culture...
June 18, 2018: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/29913124/preventive-effects-of-astragaloside-iv-and-its-active-sapogenin-cycloastragenol-on-cardiac-fibrosis-of-mice-by-inhibiting-the-nlrp3-inflammasome
#10
Yu Wan, Lu Xu, Yunxia Wang, Nuerbiya Tuerdi, Min Ye, Rong Qi
Cardiac fibrosis is a common feature of many cardiac pathophysiologic conditions. Recently, it has been shown that the activation of NLRP3 inflammasome plays an important role in the pathophysiology of cardiac fibrosis. Here, the inhibitory effects and possible mechanism of astragaloside IV (AST) and its active sapogenin cycloastragenol (CAG) on isoproterenol (ISO)-induced cardiac fibrosis were investigated. In our study, BALB/c mice were subcutaneously injected with 5mg/kg ISO for 7 consecutive days to induce cardiac fibrosis...
June 15, 2018: European Journal of Pharmacology
https://www.readbyqxmd.com/read/29910813/s100a4-is-secreted-by-alternatively-activated-alveolar-macrophages-and-promotes-activation-of-lung-fibroblasts-in-pulmonary-fibrosis
#11
Wei Zhang, Shinji Ohno, Beatrix Steer, Stephan Klee, Claudia A Staab-Weijnitz, Darcy Wagner, Mareike Lehmann, Tobias Stoeger, Melanie Königshoff, Heiko Adler
Idiopathic pulmonary fibrosis (IPF) is a devastating interstitial lung disease, characterized by damage of lung epithelial cells, excessive deposition of extracellular matrix in the lung interstitium, and enhanced activation and proliferation of fibroblasts. S100a4, also termed FSP-1 (fibroblast-specific protein-1), was previously considered as a marker of fibroblasts but recent findings in renal and liver fibrosis indicated that M2 macrophages are an important cellular source of S100a4. Thus, we hypothesized that also in pulmonary fibrosis, M2 macrophages produce and secrete S100a4, and that secreted S100a4 induces the proliferation and activation of fibroblasts...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29908958/antifungal-drug-ciclopirox-olamine-reduces-hsv-1-replication-and-disease-in-mice
#12
Kaelin M Bernier, Lynda A Morrison
Herpes simplex virus (HSV)-1 and HSV-2 cause painful blisters and shallow ulcers in exposed skin and mucosae during primary or recurrent infection. In addition, recurrent and potentially blinding HSV-1 infections of the eye afflict nearly half a million persons in the U.S. Current clinical therapies rely on nucleoside analog drugs such as acyclovir (ACV) or ganciclovir to ameliorate primary infections and reduce the frequency and duration of reactivations. However, these treatments do not fully suppress viral shedding and drug-resistant mutants develop in the eye and in vulnerable, immunosuppressed patients...
June 14, 2018: Antiviral Research
https://www.readbyqxmd.com/read/29908000/serpin-a1-and-the-modulation-of-type-i-collagen-turnover-effect-of-the-c-terminal-peptide-409-418-sa1-iii-in-human-dermal-fibroblasts
#13
Caterina Cipriani, Simona Pascarella, Fosca Errante, Beatrice Menicacci, Lucia Magnelli, Alessandra Mocali, Paolo Rovero, Lisa Giovannelli
The pharmacological modulation of collagen turnover is a strategy potentially useful in different skin conditions. The serine protease inhibitor Serpin A1 and portions of its C-terminal region have been investigated as collagen modulators. To clarify the mechanisms by which the C-terminal 409-418 peptide SA1-III increases extracellular type I collagen levels, to compare its activities range with that of the originator molecule Serpin A1, and to evaluate its efficacy in primary cultures from adult and aged human subjects...
June 16, 2018: Cell Biology International
https://www.readbyqxmd.com/read/29900391/data-regarding-the-effects-of-thrombin-and-dabigatran-inhibited-thrombin-on-protease-activated-receptor-1-and-activation-of-human-atrial-fibroblasts
#14
Paola Altieri, Maria Bertolotto, Patrizia Fabbi, Elena Sportelli, Manrico Balbi, Francesco Santini, Claudio Brunelli, Marco Canepa, Fabrizio Montecucco, Pietro Ameri
The data presented here are related to the research paper entitled "Thrombin induces protease-activated receptor 1 signaling and activation of human atrial fibroblasts and dabigatran prevents these effects" (Altieri et al., 2018) [1]. Data show that silencing of protease-activated receptor 1 (PAR1) prevents the activation of Fib isolated from atrial appendages of patients without atrial fibrillation (AF), as assessed by immunofluorescence for α-smooth muscle actin (αSMA) and Picro-Sirius red staining...
August 2018: Data in Brief
https://www.readbyqxmd.com/read/29899382/constitutive-ip-3-signaling-underlies-the-sensitivity-of-b-cell-cancers-to-the-bcl-2-ip-3-receptor-disruptor-bird-2
#15
Mart Bittremieux, Rita M La Rovere, Haidar Akl, Claudio Martines, Kirsten Welkenhuyzen, Kathia Dubron, Myriam Baes, Ann Janssens, Peter Vandenberghe, Luca Laurenti, Katja Rietdorf, Giampaolo Morciano, Paolo Pinton, Katsuhiko Mikoshiba, Martin D Bootman, Dimitar G Efremov, Humbert De Smedt, Jan B Parys, Geert Bultynck
Anti-apoptotic Bcl-2 proteins are upregulated in different cancers, including diffuse large B-cell lymphoma (DLBCL) and chronic lymphocytic leukemia (CLL), enabling survival by inhibiting pro-apoptotic Bcl-2-family members and inositol 1,4,5-trisphosphate (IP3 ) receptor (IP3 R)-mediated Ca2+ -signaling. A peptide tool (Bcl-2/IP3 R Disruptor-2; BIRD-2) was developed to abrogate the interaction of Bcl-2 with IP3 Rs by targeting Bcl-2's BH4 domain. BIRD-2 triggers cell death in primary CLL cells and in DLBCL cell lines...
June 13, 2018: Cell Death and Differentiation
https://www.readbyqxmd.com/read/29899280/efficient-knock-in-of-a-point-mutation-in-porcine-fibroblasts-using-the-crispr-cas9-gmnn-fusion-gene
#16
Max Gerlach, Theresia Kraft, Bernhard Brenner, Björn Petersen, Heiner Niemann, Judith Montag
During CRISPR/Cas9 mediated genome editing, site-specific double strand breaks are introduced and repaired either unspecific by non-homologous end joining (NHEJ) or sequence dependent by homology directed repair (HDR). Whereas NHEJ-based generation of gene knock-out is widely performed, the HDR-based knock-in of specific mutations remains a bottleneck. Especially in primary cell lines that are essential for the generation of cell culture and animal models of inherited human diseases, knock-in efficacy is insufficient and needs significant improvement...
June 13, 2018: Genes
https://www.readbyqxmd.com/read/29899108/a-kaposi-s-sarcoma-associated-herpesvirus-infection-mechanism-is-independent-of-integrins-%C3%AE-3%C3%AE-1-%C3%AE-v%C3%AE-3-and-%C3%AE-v%C3%AE-5
#17
Allison Alwan TerBush, Florianne Hafkamp, Hee Jun Lee, Laurent Coscoy
Host receptor usage by KSHV has been best studied using primary microvascular endothelial and fibroblast cells, although the virus infects a wide variety of cell types in culture and in natural infections. In these two infection models, KSHV adheres to the cell though heparan sulfate (HS) binding, then interacts with a complex of EphA2, xct, and integrins α3β1, αVβ3, αVβ5 to catalyze viral entry. We dissected this receptor complex at the genetic level with CRISPR-Cas9 to precisely determine receptor usage in two epithelial cell lines...
June 13, 2018: Journal of Virology
https://www.readbyqxmd.com/read/29896323/banding-cytogenetics-of-the-barbary-partridge-alectoris-barbara-and-the-chukar-partridge-alectoris-chukar-phasianidae-a-large-conservation-with-domestic-fowl-gallus-domesticus-revealed-by-high-resolution-chromosomes
#18
Siham Ouchia-Benissad, Kafia Ladjali-Mohammedi
The development of avian cytogenetics is significantly behind that of mammals. In fact, since the advent of cytogenetic techniques, fewer than 1500 karyotypes have been established. The Barbary partridge Alectoris barbara Bonnaterre, 1790 is a bird of economic interest but its genome has not been studied so far. This species is endemic to North Africa and globally declining. The Chukar partridge Alectoris chukar Gray, 1830 is an introduced species which shares the same habitat area as the Barbary partridge and so there could be introgressive hybridisation...
2018: Comparative Cytogenetics
https://www.readbyqxmd.com/read/29896321/in-vitro-characterization-of-variable-porosity-wound-dressing-with-anti-scar-properties
#19
Collynn F Woeller, Aubrey Woodroof, Patrick S Cottler, Stephen J Pollock, Constantine G Haidaris, Richard P Phipps
Introduction: New options are needed to improve wound healing while preventing excessive scar formation. Temporary primary dressings are important options in topical wound management that allow the natural healing process. Methods: We evaluated a novel primary dressing consisting of a biosynthetic, variable porosity, matrix-containing gelatin and Aloe Vera extract and a derivative dressing coated with the anti-scarring agent salinomycin for their ability to promote cell growth, reduce myofibroblast formation, and regulate cytokine production...
2018: Eplasty
https://www.readbyqxmd.com/read/29895976/an-essential-role-for-wnt-%C3%AE-catenin-signaling-in-mediating-hypertensive-heart-disease
#20
Yue Zhao, Chunhong Wang, Cong Wang, Xue Hong, Jinhua Miao, Yulin Liao, Lili Zhou, Youhua Liu
Activation of the renin-angiotensin system (RAS) is associated with hypertension and heart disease. However, how RAS activation causes cardiac lesions remains elusive. Here we report the involvement of Wnt/β-catenin signaling in this process. In rats with chronic infusion of angiotensin II (Ang II), eight Wnt ligands were induced and β-catenin activated in both cardiomyocytes and cardiac fibroblasts. Blockade of Wnt/β-catenin signaling by small molecule inhibitor ICG-001 restrained Ang II-induced cardiac hypertrophy by normalizing heart size and inhibiting hypertrophic marker genes...
June 12, 2018: Scientific Reports
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