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https://www.readbyqxmd.com/read/29928182/a-novel-mgp-gene-mutation-causing-keutel-syndrome-in-a-brazilian-patient
#1
Eduardo Perrone, Kelin Chen, Marco Ramos, Maria Fernanda Milanezi, Viviane Nakano, Ariane Falconi, Juliana Silva, Jamille Campos, Celia M C Silva, Joao B O Filho, Ana B A Perez
Keutel syndrome is caused by mutations in the matrix gamma-carboxyglutamic acid ( MGP ) gene (OMIM 154870) and is inherited in an autosomal recessive fashion. It is characterized by brachydactyly, pulmonary artery stenosis, a distinctive facial phenotype, and cartilage calcification. To date, only 36 cases have been reported worldwide. We describe clinical and molecular findings of the first Brazilian patient with Keutel syndrome. Keutel syndrome was suspected based on clinical and morphological evaluation, so we sequenced the MGP gene using the TruSight One Sequencing Panel (Illumina)...
May 2018: Molecular Syndromology
https://www.readbyqxmd.com/read/29903718/calcitriol-accelerates-vascular-calcification-irrespective-of-vitamin-k-status-in-a-rat-model-of-ckd-with-hyperphosphatemia-and-secondary-hyperparathyroidism
#2
Kristin M McCabe, Jason G Zelt, Martin Kaufmann, Kimberly Laverty, Emilie Ward, Henry Barron, Glenville Jones, Michael A Adams, Rachel M Holden
Patients with chronic kidney disease have a markedly increased risk for developing cardiovascular disease. Non-traditional risk factors, such as increased phosphate retention, and deficiencies in vitamins D and K metabolism, likely play key roles in the development of vascular calcification during CKD progression. Calcitriol (1,25-(OH)2-D3) is a key transcriptional regulator of Matrix Gla protein (MGP), a vitamin K dependent protein that inhibits vascular calcification. The objective of this study was to determine if calcitriol treatment could inhibit the development of vascular calcification and if this inhibition was dependent on vitamin K status in a rat model of CKD...
June 14, 2018: Journal of Pharmacology and Experimental Therapeutics
https://www.readbyqxmd.com/read/29888653/decreased-expression-of-cell-adhesion-genes-in-cancer-stem-like-cells-isolated-from-primary-oral-squamous-cell-carcinomas
#3
Amrendra Mishra, Harshini Sriram, Pinal Chandarana, Vivek Tanavde, Rekha V Kumar, Ashok Gopinath, Raman Govindarajan, S Ramaswamy, Subhashini Sadasivam
The goal of this study was to isolate cancer stem-like cells marked by high expression of CD44, a putative cancer stem cell marker, from primary oral squamous cell carcinomas and identify distinctive gene expression patterns in these cells. From 1 October 2013 to 4 September 2015, 76 stage III-IV primary oral squamous cell carcinoma of the gingivobuccal sulcus were resected. In all, 13 tumours were analysed by immunohistochemistry to visualise CD44-expressing cells. Expression of CD44 within The Cancer Genome Atlas-Head and Neck Squamous Cell Carcinoma RNA-sequencing data was also assessed...
May 2018: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
https://www.readbyqxmd.com/read/29880866/mesenchymal-stromal-cells-derived-matrix-gla-protein-contribute-to-the-alleviation-of-experimental-colitis
#4
Yuan Feng, Yan Liao, Weijun Huang, Xingqiang Lai, Jing Luo, Cong Du, Junyi Lin, Zhongyuan Zhang, Dongbo Qiu, Qiuli Liu, Huiyong Shen, Andy Peng Xiang, Qi Zhang
Crohn's disease (CD) is a chronic inflammatory bowel disease that is difficult to treat. However, previous preclinical and clinical studies have shown that mesenchymal stromal cells (MSCs) are a promising therapeutic approach, whereas the exact underlying molecular mechanisms of MSCs in treating CD remain unclear. Furthermore, the heterogeneity of MSCs, as well as the in vivo microenvironments may influence the therapeutic efficacy. In our previous study, we found that a subpopulation of mouse MSCs with a high expression of matrix Gla protein (MGP), one of the members of vitamin K-dependent protein family, possessed better immunoregulatory properties...
June 7, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29850574/bone-morphogenetic-proteins-2-4-are-upregulated-during-the-early-development-of-vascular-calcification-in-chronic-kidney-disease
#5
Xiao Wei, Weihua Wu, Li Li, Jiaru Lin, Qi Liu, Linwang Gan, Santao Ou
Vascular calcification is a main cause of increased cardiovascular morbidity and mortality in chronic kidney disease (CKD) patients. This study aimed to investigate the role of the bone morphogenetic protein (BMP) signaling pathway in the early development of vascular calcification in CKD. A CKD vascular calcification rat model was established by providing rats with a 1.8% high-phosphorus diet and an intragastric administration of 2.5% adenine suspension. The kidney and aortic pathologies were analyzed. Blood biochemical indicators, serum BMP-2 and BMP-4 levels, and aortic calcium content were determined...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29797901/-community-structure-of-soil-mites-under-quercus-variabilis-forests-in-small-watershed-of-karst-area-guizhou-china
#6
Hu Chen, Dao Chao Jin, Yan Zhang
To analyze the differences of community structure of soil mites between the rocky desertification environment and karst beech forests, we investigated the soil mites of Quercus variabilis forest in the Chaoying small watershed of Guizhou Province. Soil mites from 83 genera, 54 families, and 3 orders were collected in the four investigations of 2014. We analyzed the differences of genus numbers, individual numbers, individual density, Shannon diversity index (H), Margalef richness index (SR), Pielou evenness index (J), maturity index (MI) of predatory soil mites (Mesostigmata: Gamasina), community structure of the oribatid mites (MGP) and nutritional structure of mite...
May 2018: Ying Yong Sheng Tai Xue Bao, the Journal of Applied Ecology
https://www.readbyqxmd.com/read/29792498/diagnostic-accuracy-of-a-universal-screening-multiple-gating-procedure-a-replication-study
#7
Stephen P Kilgus, Nathaniel P von der Embse, Crystal N Taylor, Michael P Van Wie, Wesley A Sims
The purpose of this diagnostic accuracy study was to evaluate the sensitivity and specificity (among other indicators) of three universal screening approaches, including the Social, Academic, and Emotional Behavior Risk Screener (SAEBRS), a SAEBRS-based teacher nomination tool, and a multiple gating procedure (MGP). Each screening approach was compared to the BASC-2 Behavioral and Emotional Screening System (BESS), which served as a criterion indicator of student social-emotional and behavioral risk. All data were collected in a concurrent fashion...
May 24, 2018: School Psychology Quarterly
https://www.readbyqxmd.com/read/29788226/circulating-dp-ucmgp-is-associated-with-kidney-dysfunction-and-arterial-stiffness
#8
Houry Puzantian, Scott R Akers, Garrett Oldland, Khuzaima Javaid, Rachana Miller, Yueya Ge, Bilal Ansari, Jonathan Lee, Arpita Suri, Zeba Hasmath, Raymond Townsend, Julio A Chirinos
Background: Large artery stiffening is increased in advanced CKD, but likely develops progressively in earlier stages of CKD. Active matrix Gla-Protein (MGP) is a potent vitamin-K dependent inhibitor of vascular calcification. A recent animal model demonstrated intrinsic abnormalities in vitamin K metabolism even in early CKD, but whether early human CKD is associated with vascular vitamin K deficiency is unknown. Methods: We enrolled 137 adults without HF with varying degrees of renal function: normal eGFR (>90 mL/min; n=59), mildly reduced eGFR (stage 2 CKD: eGFR = 60-89 mL/min; n=53) or at least moderately reduced eGFR (stage 3-5 CKD; eGFR<60 ml/min; n=25)...
May 17, 2018: American Journal of Hypertension
https://www.readbyqxmd.com/read/29777408/genetic-association-analysis-of-osteopontin-and-matrix-gla-protein-genes-polymorphisms-with-primary-knee-osteoarthritis-in-mexican-population
#9
Verónica Marusa Borgonio-Cuadra, Norma Celia González-Huerta, Emma Xochitl Rojas-Toledo, Eugenio Morales-Hernández, Nonanzit Pérez-Hernández, José Manuel Rodríguez-Pérez, Carlos Alfonso Tovilla-Zárate, Thelma Beatriz González-Castro, Yazmín Hernández-Díaz, María Lilia López-Narváez, Antonio Miranda-Duarte
Primary osteoarthritis (OA) is a complex entity in which several loci related to different molecular pathways or classes of molecules are associated with its development as demonstrated through genetic association studies. Genes involved in bone formation and mineralization, such as osteopontin (OPN) and Matrix Gla protein (MGP), could also be related with OA. The aim of this study was to evaluate the association between the genetic variants of OPN and MGP with primary knee osteoarthritis in a Mexican population...
May 18, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29776661/the-role-of-intra-napl-diffusion-on-mass-transfer-from-mgp-residuals
#10
Saeid Shafieiyoun, Neil R Thomson
An experimental and computational study was performed to investigate the role of multi-component intra-NAPL diffusion on NAPL-water mass transfer. Molecular weight and the NAPL component concentrations were determined to be the most important parameters affecting intra-NAPL diffusion coefficients. Four NAPLs with different viscosities but the same quantified mass were simulated. For a spherical NAPL body, a combination of NAPL properties and interphase mass transfer rate can result in internal diffusion limitations...
April 24, 2018: Journal of Contaminant Hydrology
https://www.readbyqxmd.com/read/29764156/nonlocal-kinetic-energy-functionals-by-functional-integration
#11
Wenhui Mi, Alessandro Genova, Michele Pavanello
Since the seminal studies of Thomas and Fermi, researchers in the Density-Functional Theory (DFT) community are searching for accurate electron density functionals. Arguably, the toughest functional to approximate is the noninteracting kinetic energy, Ts [ρ], the subject of this work. The typical paradigm is to first approximate the energy functional and then take its functional derivative, δTs [ρ]δρ(r), yielding a potential that can be used in orbital-free DFT or subsystem DFT simulations. Here, this paradigm is challenged by constructing the potential from the second-functional derivative via functional integration...
May 14, 2018: Journal of Chemical Physics
https://www.readbyqxmd.com/read/29726090/gene-expression-signatures-prognostic-for-relapse-in-stage-i-testicular-germ-cell-tumors-tgct
#12
Jeremy Lewin, Laleh Soltan Ghoraie, Philippe L Bedard, Robert J Hamilton, Peter Chung, Malcolm Moore, Michael A S Jewett, Lynn Anson-Cartwright, Carl Virtanen, Neil Winegarden, Julie Tsao, Padraig Warde, Joan Sweet, Benjamin Haibe-Kains, Aaron R Hansen
OBJECTIVES: To identify differentially expressed genes between relapsed (R) and non-relapsed (NR) clinical stage I (CSI) testicular germ cell tumor (TGCT). MATERIALS AND METHODS: We reviewed patients with CSI non-seminoma(NS) and seminoma(S) from an institutional database (2000-2012) managed by active surveillance. NR-NS and NR-S patients were defined as being relapse free after 2 and 3 years of surveillance respectively. RNA extraction and gene expression analysis was performed on archival primary tumor samples and Gene-Set-Enrichment-Analysis (GSEA) was conducted in order to identify differentiating biological pathways...
May 3, 2018: BJU International
https://www.readbyqxmd.com/read/29722960/a-natural-triterpenoid-tailored-phosphate-in-situ-reduction-of-heavy-metals-spontaneously-to-generate-electrochemical-hybrid-gels
#13
Yuxia Gao, Jie Hao, Qiang Yan, Fengpei Du, Yong Ju, Jun Hu
In this work we reported a biocompatible nature product-based soft material which could convert heavy metals to nanoparticles (NPs) in situ spontaneously in a simple step. We have designed and synthesized a natural triterpenoid-tailored phosphate (MGP), and this amphiphilic MGP could form the stable hydrogel and extract gold salt from water, followed by spontaneous in situ Au nanoparticles (AuNPs) formation without external reductants. Notably, the AuNPs were mainly localized on nanofibers instead of gel cavities, and the resulting MGP-AuNPs hybrid gel exhibited attractive electrocatalytic and conductive properties...
May 3, 2018: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/29689070/fti-277-inhibits-smooth-muscle-cell-calcification-by-up-regulating-pi3k-akt-signaling-and-inhibiting-apoptosis
#14
Arvind Ponnusamy, Smeeta Sinha, Gareth D Hyde, Samantha J Borland, Rebecca F Taylor, Emma Pond, Heather J Eyre, Colette A Inkson, Andrew Gilmore, Nick Ashton, Philip A Kalra, Ann E Canfield
BACKGROUND: Vascular calcification is associated with increased cardiovascular morbidity and mortality in patients with atherosclerosis, diabetes and chronic kidney disease. However, no viable treatments for this condition have been identified. This study aimed to determine whether farnesyl transferase inhibitors (FTIs) can reduce vascular calcification and the mechanism by which this reduction occurs. RESULTS: We demonstrate that FTI-277 significantly inhibits phosphate-induced mineral deposition by vascular smooth muscle cells (VSMC) in vitro, prevents VSMC osteogenic differentiation, and increases mRNA expression of matrix Gla protein (MGP), an inhibitor of mineralization...
2018: PloS One
https://www.readbyqxmd.com/read/29584693/is-matrix-gla-protein-associated-with-vascular-calcification-a-systematic-review
#15
REVIEW
Hilary Barrett, Mary O'Keeffe, Eamon Kavanagh, Michael Walsh, Eibhlís M O'Connor
Specific patient cohorts are at increased risk of vascular calcification. Functional matrix-gla protein (MGP), a tissue-derived vitamin K dependent protein, is reported to be an important inhibitor of vascular calcification and may have clinical potential to modify the progression of vascular calcification through regulation of functional MGP fractions. This systematic review examines twenty-eight studies which assess the relationship between circulating protein expressions of MGP species and vascular calcification in different arterial beds...
March 27, 2018: Nutrients
https://www.readbyqxmd.com/read/29582552/efficacy-and-tolerability-of-magnesium-plus-protein-for-managing-hypomagnesemia-in-pediatric-kidney-transplant-patients
#16
Teresa V Lewis, Stephen Neely, Martin A Turman
We sought to investigate whether magnesium oxide bound to soy protein (MGP) increases serum magnesium concentrations with less diarrhea compared to commonly prescribed magnesium salts. Subjects were switched to MGP at a near-equivalent daily elemental magnesium dose. Mean serum magnesium levels were compared. If magnesium levels remained <1.7 mg/dL after switching to MGP, subjects were enrolled into Part 2 and received a one-time MGP dose adjustment. The MGP daily dose was increased by 266 mg. For both parts 1 and 2, subjects recorded the number and quality of their stools to assess gastrointestinal (GI) tolerability of MGP...
March 27, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29563538/ucma-grp-inhibits-phosphate-induced-vascular-smooth-muscle-cell-calcification-via-smad-dependent-bmp-signalling
#17
Brecht A Willems, Malgorzata Furmanik, Marjolein M J Caron, Martijn L L Chatrou, Dennis H M Kusters, Tim J M Welting, Michael Stock, Marta S Rafael, Carla S B Viegas, Dina C Simes, Cees Vermeer, Chris P M Reutelingsperger, Leon J Schurgers
Vascular calcification (VC) is the process of deposition of calcium phosphate crystals in the blood vessel wall, with a central role for vascular smooth muscle cells (VSMCs). VC is highly prevalent in chronic kidney disease (CKD) patients and thought, in part, to be induced by phosphate imbalance. The molecular mechanisms that regulate VC are not fully known. Here we propose a novel role for the mineralisation regulator Ucma/GRP (Upper zone of growth plate and Cartilage Matrix Associated protein/Gla Rich Protein) in phosphate-induced VSMC calcification...
March 21, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29561783/bicuspid-aortic-valve-stenosis-and-the-effect-of-vitamin-k2-on-calcification-using-18-f-sodium-fluoride-positron-emission-tomography-magnetic-resonance-the-basik2-rationale-and-trial-design
#18
Frederique E C M Peeters, Manouk J W van Mourik, Steven J R Meex, Jan Bucerius, Simon M Schalla, Suzanne C Gerretsen, Casper Mihl, Marc R Dweck, Leon J Schurgers, Joachim E Wildberger, Harry J G M Crijns, Bas L J H Kietselaer
BASIK2 is a prospective, double-blind, randomized placebo-controlled trial investigating the effect of vitamin K2 (menaquinone-7;MK7) on imaging measurements of calcification in the bicuspid aortic valve (BAV) and calcific aortic valve stenosis (CAVS). BAV is associated with early development of CAVS. Pathophysiologic mechanisms are incompletely defined, and the only treatment available is valve replacement upon progression to severe symptomatic stenosis. Matrix Gla protein (MGP) inactivity is suggested to be involved in progression...
March 21, 2018: Nutrients
https://www.readbyqxmd.com/read/29552534/status-of-single-nucleotide-polymorphism-of-matrix-gla-protein-gene-rs4236-in-nephrolithiasis-a-preliminary-study-in-indian-population
#19
Anandan Murugesan, Lakshman Kumar, P Janarthanan
Background: Urine is a supersaturated solution with solutes in very high concentration. Matrix gla protein (MGP) is one of the proteins involved in inhibition of extracellular calcification. Among the various polymorphisms studied, polymorphism of SNP rs4236 in the MGP gene is found to have association with nephrolithiasis. We evaluated the significance of SNP rs4236 polymorphism in nephrolithiasis among the Indian population. Materials and Methods: The study was conducted in 2013 and 2014 among fifty participants...
January 2018: International Journal of Applied and Basic Medical Research
https://www.readbyqxmd.com/read/29529056/epidemiological-and-histological-findings-implicate-matrix-gla-protein-in-diastolic-left-ventricular-dysfunction
#20
Fang-Fei Wei, Sander Trenson, Pierre Monney, Wen-Yi Yang, Menno Pruijm, Zhen-Yu Zhang, Yassine Bouatou, Qi-Fang Huang, Belen Ponte, Pierre-Yves Martin, Lutgarde Thijs, Tatiana Kuznetsova, Karel Allegaert, Stefan Janssens, Cees Vermeer, Peter Verhamme, Michel Burnier, Murielle Bochud, Georg Ehret, Jan A Staessen
OBJECTIVES: A novel paradigm of diastolic left ventricular (LV) dysfunction proposes involvement of the cardiac microvasculature. Vitamin K dependent matrix Gla protein (MGP) plays a role in preserving microcirculatory integrity. We hypothesized that LV filling pressure-a measure of diastolic LV dysfunction-increases with higher plasma level of inactive desphospho-uncarboxylated MGP (dp-ucMGP). We also studied the distribution of active and inactive MGP in human myocardium. METHODS: We measured echocardiographic diastolic LV function and plasma dp-ucMGP (ELISA) in 668 Flemish and for replication in 386 Swiss...
2018: PloS One
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