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AdamTS 13

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https://www.readbyqxmd.com/read/27878664/diagnosis-and-management-of-acquired-thrombotic-thrombocytopenic-purpura-in-southeast-china-a-single-center-experience-of-60-cases
#1
Xinping Zhou, Xingnong Ye, Yanling Ren, Chen Mei, Liya Ma, Jiansong Huang, Weilai Xu, Juying Wei, Li Ye, Wenyuan Mai, Wenbin Qian, Haitao Meng, Jie Jin, Hongyan Tong
Acquired thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening thrombotic microangiopathy. This study aimed to provide a profile of the diagnosis and management of patients with acquired TTP collected in 10 years in a single center in southeast China. A total of 60 patients diagnosed with acute acquired TTP from March 2005 to August 2015 were enrolled. Among the 60 patients, 52 patients presented with their first episodes, and eight patients had two or more episodes. The median age at presentation was 49 (range, 17 to 78) years with a female predominance (male:female ratio, 1:1...
November 23, 2016: Frontiers of Medicine
https://www.readbyqxmd.com/read/27863298/butein-inhibits-il-1%C3%AE-induced-inflammatory-response-in-human-osteoarthritis-chondrocytes-and-slows-the-progression-of-osteoarthritis-in-mice
#2
Wenhao Zheng, Hui Zhang, Yonglong Jin, Quan Wang, Linwei Chen, Zhenhua Feng, Hua Chen, Yaosen Wu
Osteoarthritis (OA) is a progressive degenerative disease characterized by irreversible articular cartilage destruction. Butein, a polyphenolic compound isolated from the stem bark of cashews and Rhus verniciflua Stokes, has been reported to have anti-inflammatory effects. This study aimed to assess the effect of butein on human OA chondrocytes and mice OA models induced by destabilization of the medial meniscus (DMM). In vitro, human OA chondrocytes were pretreated with butein at 10, 50μM and subsequently stimulated with IL-1β (10ng/ml) for 24h...
November 14, 2016: International Immunopharmacology
https://www.readbyqxmd.com/read/27807630/-role-of-plasmapheresis-and-immunoadsorption-in-salvage-therapy-of-rheumatological-diseases
#3
M Boser, J T Kielstein
Many rheumatological diseases are either caused by specific known proteins, such as antibodies or mediated by a plethora of cytokines. Both the unspecific immunosuppressive therapy and the specific action of biologics usually require time to be effective; therefore, extracorporeal forms of treatment are increasingly being employed in severe forms of rheumatological diseases as well as in patients who cannot tolerate pharmacological treatment or where the risk of pharmacological treatment may outweigh the potential benefits...
December 2016: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/27792110/role-of-sirt1-plays-in-nucleus-pulposus-cells-and-intervertebral-disc-degeneration
#4
Ji Guo, Minghao Shao, Feizhou Lu, Jianyuan Jiang, Xinlei Xia
STUDY DESIGN: Experimental in vivo and in vitro study of intervertebral disc (IVD) degeneration and the mechanism exploration. OBJECTIVE: This report aims to verify the expression of Sirt1 in IVD degeneration of different grades and explore its potential mechanism in human nucleus pulposus cells. SUMMARY OF BACKGROUND DATA: Silent mating type information regulator 2 homolog 1 (Sirt1) has draw immense attention because of its influence on a variety of aging-related diseases...
October 25, 2016: Spine
https://www.readbyqxmd.com/read/27789465/systemic-inhibition-of-il-6-stat3-signalling-protects-against-experimental-osteoarthritis
#5
Augustin Latourte, Chahrazad Cherifi, Jérémy Maillet, Hang-Korng Ea, Wafa Bouaziz, Thomas Funck-Brentano, Martine Cohen-Solal, Eric Hay, Pascal Richette
OBJECTIVE: To investigate the impact of systemic inhibition of interleukin 6 (IL-6) or signal transducer and activator of transcription (Stat3) in an experimental model of osteoarthritis (OA). METHODS: Expression of major catabolic and anabolic factors of cartilage was determined in IL-6-treated mouse chondrocytes and cartilage explants. The anti-IL-6-receptor neutralising antibody MR16-1 was used in the destabilisation of the medial meniscus (DMM) mouse model of OA...
October 27, 2016: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27781079/atypical-hemolytic-uremic-syndrome-secondary-to-lupus-nephritis-responsive-to-eculizumab
#6
Alexander G Raufi, Shruti Scott, Omar Darwish, Kevin Harley, Kanwarpal Kahlon, Sheetal Desai, Yuxin Lu, Minh-Ha Tran
Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) - a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage...
September 28, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27736317/a-three-dimensional-chondrocyte-macrophage-coculture-system-to-probe-inflammation-in-experimental-osteoarthritis
#7
Satyavrata Samavedi, Patricia Diaz-Rodriguez, Joshua D Erndt-Marino, Mariah S Hahn
The goal of the present study was to develop a fully three-dimensional (3D) coculture system that would allow for systematic evaluation of the interplay between activated macrophages (AMs) and chondrocytes in osteoarthritic disease progression and treatment. Toward this end, our coculture system was first validated against existing in vitro osteoarthritis models, which have generally cultured healthy normal chondrocytes (NCs)-in two-dimensional (2D) or 3D-with proinflammatory AMs in 2D. In this work, NCs and AMs were both encapsulated within poly(ethylene glycol) diacrylate hydrogels to mimic the native 3D environments of both cell types within the osteoarthritic joint...
November 18, 2016: Tissue Engineering. Part A
https://www.readbyqxmd.com/read/27717422/left-ventricular-assist-device-design-reduces-von-willebrand-factor-degradation-a-comparative-study-between-the-heartmate-ii-and-the-evaheart-left-ventricular-assist-system
#8
Carlo R Bartoli, Jooeun Kang, David Zhang, Jessica Howard, Michael Acker, Pavan Atluri, Tadashi Motomura
BACKGROUND: Supraphysiologic shear stress from continuous-flow left ventricular assist devices (LVADs) accelerates von Willebrand factor (vWF) degradation and predisposes patients to nonsurgical bleeding. It is unknown whether unique design characteristics of LVADs differentially affect vWF degradation. We tested the hypothesis that the centrifugal-flow EVAHEART (Evaheart, Houston, TX) left ventricular assist system (LVAS), which was designed to minimize shear stress (low operational revolutions per minute [rpm], larger flow gaps, low shear stress, flat H-Q curve), reduced vWF degradation versus the axial-flow HeartMate II (Thoratec, Pleasanton, CA) LVAD...
October 4, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27699833/effects-of-cell-type-and-configuration-on-anabolic-and-catabolic-activity-in-3d-coculture-of-mesenchymal-stem-cells-and-nucleus-pulposus-cells
#9
Ann Ouyang, Alec E Cerchiari, Xinyan Tang, Ellen Liebenberg, Tamara Alliston, Zev J Gartner, Jeffrey C Lotz
Tissue engineering constructs to treat intervertebral disc degeneration must adapt to the hypoxic and inflammatory degenerative disc microenvironment. The objective of this study was to determine the effects of two key design factors, cell type and cell configuration, on the regenerative potential of nucleus pulposus cell (NPC) and mesenchymal stem cell (MSC) constructs. Anabolic and catabolic activity was quantified in constructs of varying cell type (NPCs, MSCs, and a 50:50 coculture) and varying configuration (individual cells and micropellets)...
October 4, 2016: Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society
https://www.readbyqxmd.com/read/27658702/oxygen-tension-modulates-the-effects-of-tnf%C3%AE-in-compressed-chondrocytes
#10
R K Tilwani, S Vessillier, B Pingguan-Murphy, D A Lee, D L Bader, T T Chowdhury
OBJECTIVE AND DESIGN: Oxygen tension and biomechanical signals are factors that regulate inflammatory mechanisms in chondrocytes. We examined whether low oxygen tension influenced the cells response to TNFα and dynamic compression. MATERIALS AND METHODS: Chondrocyte/agarose constructs were treated with varying concentrations of TNFα (0.1-100 ng/ml) and cultured at 5 and 21 % oxygen tension for 48 h. In separate experiments, constructs were subjected to dynamic compression (15 %) and treated with TNFα (10 ng/ml) and/or L-NIO (1 mM) at 5 and 21 % oxygen tension using an ex vivo bioreactor for 48 h...
September 22, 2016: Inflammation Research: Official Journal of the European Histamine Research Society ... [et Al.]
https://www.readbyqxmd.com/read/27646982/hedgehog-signal-expression-in-articular-cartilage-of-rat-temporomandibular-joint-and-association-with-adjuvant-induced-osteoarthritis
#11
Ting Xu, Gaoli Xu, Zhiyuan Gu, Huiling Wu
OBJECTIVE: The aim of this study was to investigate the changes in hedgehog (Hh) expression and its possible effects on cartilage degeneration in adjuvant-induced temporomandibular joint osteoarthritis (TMJOA) of rats. METHODS: Forty-eight male Sprague-Dawley rats were randomly divided into experimental osteoarthritis (OA) and sham control groups. The bilateral TMJs of six rats from each group were harvested at three, seven, 14, and 28 days. Histological changes in condylar cartilage were assessed by hematoxylin and eosin, toluidine blue, and safranin O staining...
September 20, 2016: Journal of Oral Pathology & Medicine
https://www.readbyqxmd.com/read/27638769/adamts18-deletion-results-in-distinct-developmental-defects-and-provides-a-model-for-congenital-disorders-of-lens-lung-and-female-reproductive-tract-development
#12
Dalya Ataca, Marian Caikovski, Alessandra Piersigilli, Alexandre Moulin, Charaf Benarafa, Sarah E Earp, Yakir Guri, Corinne Kostic, Yvan Arsenivic, Raija Soininen, Suneel S Apte, Cathrin Brisken
The ADAMTS family comprises 19 secreted metalloproteinases that cleave extracellular matrix components and have diverse functions in numerous disease and physiological contexts. A number of them remain 'orphan' proteases and among them is ADAMTS18, which has been implicated in developmental eye disorders, platelet function and various malignancies. To assess in vivo function of ADAMTS18, we generated a mouse strain with inactivated Adamts18 alleles. In the C57Bl6/Ola background, Adamts18-deficient mice are born in a normal Mendelian ratio, and are viable but show a transient growth delay...
November 15, 2016: Biology Open
https://www.readbyqxmd.com/read/27622772/microangiopathic-hemolytic-anemia-due-to-adamts-13-loss-in-idiopathic-systemic-capillary-leak-syndrome
#13
D C Moreira, C J Ng, R Quinones, X Liang, D W Chung, J Di Paola
: Essentials Idiopathic systemic capillary leak syndrome (SCLS) is characterized by episodes of vascular leakage. We present the case of a patient with SCLS who developed microangiopathic hemolytic anemia (MAHA). We propose that this anemia is the result of ADAMTS-13 loss in the third-space fluid. This suggests that MAHA can occur in patients with significant extravasation of proteins. SUMMARY: Idiopathic systemic capillary leak syndrome (SCLS) is a rare process characterized by acute and recurrent episodes of vascular leakage with severe hypotension, hypoalbuminemia, hemoconcentration and edema...
September 13, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27621680/hematopoietic-cell-transplantation-associated-thrombotic-microangiopathy-a-review-of-pathophysiology-diagnosis-and-treatment
#14
REVIEW
Joseph Rosenthal
Transplant-associated thrombotic microangiopathy (TA-TMA) is a multifactorial disorder caused by systemic vascular endothelial injury that can be triggered by several mechanisms during the transplant process. Thrombotic microangiopathy may affect multiple systems and occurs in ~30% of patients undergoing hematopoietic stem cell transplantation. A subgroup of patients with thrombotic microangiopathy develop TA-TMA, and the other may develop other thrombotic microangiopathic disorders such as thrombotic thrombocytopenic purpura, a condition with similar finding but different pathophysiology involving ADAMTS-13...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27615609/interleukin-1%C3%AE-induces-focal-degradation-of-biglycan-and-tissue-degeneration-in-an-in-vitro-ovine-meniscal-model
#15
Emily Fuller, Christopher B Little, James Melrose
We have developed an ovine meniscal explant model where the focal degradative events leading to characteristic fragmentation patterns of biglycan in human OA of the knee and hip, and evident in animal models of knee OA and IVD degeneration are reproduced in culture. Lateral and medial menisci were dissected into outer, mid and inner zones and established in explant culture±IL-1 (10ng/ml). The biglycan species present in conditioned media samples and in GuHCl extracts of tissues were examined by Western blotting using two C-terminal antibodies PR-85 and EF-Bgn...
September 9, 2016: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/27613177/evaluation-of-von-willebrand-factor-and-adamts-13-antigen-and-activity-levels-in-sickle-cell-disease-patients-in-kuwait
#16
Anwar Al-Awadhi, Adekunle Adekile, Rajaa Marouf
Sickle cell disease (SCD) is a severe form of hemolytic anemia characterized by chronic hemolysis and is associated with increased thrombotic risk. Elevated von Willebrand factor (vWF) levels in SCD have been attributed to increased secretion and impaired processing by its cleaving protease ADAMTS-13. In this study we measured vWF and ADAMTS-13 antigen and activity levels in our SCD patients. Hematological and biochemical parameters for 59 SCD patients (20 children and 39 adults) were analyzed and compared to 59 age- and sex-matched controls...
September 9, 2016: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/27582494/engineered-tissue-inhibitor-of-metalloproteinases-3-variants-resistant-to-endocytosis-have-prolonged-chondroprotective-activity
#17
Christine M Doherty, Robert Visse, Deendayal Dinakarpandian, Dudley K Strickland, Hideaki Nagase, Linda Troeberg
Tissue inhibitor of metalloproteinases-3 (TIMP-3) is a central inhibitor of matrix-degrading and sheddase families of metalloproteinases. Extracellular levels of the inhibitor are regulated by the balance between its retention on the extracellular matrix and its endocytic clearance by the scavenger receptor low density lipoprotein receptor-related protein 1 (LRP1). Here, we used molecular modeling to predict TIMP-3 residues potentially involved in binding to LRP1 based on the proposed LRP1 binding motif of 2 lysine residues separated by about 21 Å and mutated the candidate lysine residues to alanine individually and in pairs...
October 14, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27559008/low-adamts-13-activity-and-the-risk-of-coronary-heart-disease-a-prospective-cohort-study-the-rotterdam-study
#18
M A H Sonneveld, M Kavousi, M A Ikram, A Hofman, O L Rueda Ochoa, P L Turecek, O H Franco, F W G Leebeek, M P M de Maat
: Essentials An association between ADAMTS-13 and coronary heart disease (CHD) has been suggested. 5688 participants ≥ 55 years from the Rotterdam Study without a history of CHD were included. Over a median follow-up time of 9.7 years, 456 individuals suffered from CHD. Low ADAMTS-13 activity was associated with an increased CHD risk. SUMMARY: Background The metalloprotease ADAMTS-13 cleaves high-molecular-weight von Willebrand factor multimers into smaller, less procoagulant forms...
November 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27545639/-efficacy-of-eculizumab-in-a-case-of-pregnancy-associated-ahus
#19
Raffaella Cravero, Gianluigi Ardissino, Umberto Colageo, Piernicola Staffa, Elena Bruschetta, Serena Maroni, Eirini Karvela, Ermanno Spagarino, Velia Ruggeri, Roberto Jura
Pregnancy-associated thrombotic microangiopathy (TMA) is a rare condition, but it is burdened by a significant perinatal and maternal morbidity as well as mortality. We describe the case of a 33-year-old woman, who developed a TMA at the 36th week of gestation characterized by increased LDH, haptoglobin consumption, schistocytes, thrombocytopenia and acute renal failure requiring dialysis. There were not gestational hypertension nor proteinuria until the day of hospitalization. ADAMTS 13 deficiency was ruled out and the patient did not have diarrhea...
July 2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/27539752/the-inhibition-of-ezh2-ameliorates-osteoarthritis-development-through-the-wnt-%C3%AE-catenin-pathway
#20
Linwei Chen, Yaosen Wu, Yan Wu, Ye Wang, Liaojun Sun, Fangcai Li
The purpose of our study was to elucidate the role of the histone methyltransferase enhancer of zeste homologue 2 (EZH2) in the pathophysiology of osteoarthritis (OA) and to develop a strategy to modulate EZH2 activity for OA treatment. The expression of EZH2 in normal and OA human cartilage was compared by western blotting. The effect of EZH2 overexpression and inhibition on chondrocyte hypertrophy related gene expression was examined by real-time PCR, and histone methylation on the promoter of the Wnt inhibitor SFRP1 was analyzed using a chromatin immunoprecipitation (ChIP) PCR...
2016: Scientific Reports
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