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https://www.readbyqxmd.com/read/29779902/molecular-genetics-and-metabolism-special-edition-diagnosis-diagnosis-and-prognosis-of-mucopolysaccharidosis-iva
#1
REVIEW
Hira Peracha, Kazuki Sawamoto, Lauren Averill, Heidi Kecskemethy, Mary Theroux, Mihir Thacker, Kyoko Nagao, Christian Pizarro, William Mackenzie, Hironori Kobayashi, Seiji Yamaguchi, Yasuyuki Suzuki, Kenji Orii, Tadao Orii, Toshiyuki Fukao, Shunji Tomatsu
Mucopolysaccharidosis IVA (MPS IVA, Morquio A syndrome) is an autosomal recessive disorder caused by the deficiency of N-acetylgalactosamine-6-sulfate sulfatase. Deficiency of this enzyme leads to the accumulation of specific glycosaminoglycans (GAGs), chondroitin-6-sulfate (C6S) and keratan sulfate (KS), which are mainly synthesized in the cartilage. Therefore, the substrates are stored primarily in the cartilage and its extracellular matrix (ECM), leading to a direct impact on bone development and successive systemic skeletal spondylepiphyseal dysplasia...
May 15, 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29729279/gc-ms-determination-of-nitrous-anhydrase-activity-of-bovine-and-human-carbonic-anhydrase-ii-and-iv
#2
Erik Hanff, Maximilian Zinke, Anke Böhmer, Janine Niebuhr, Mirja Maassen, Volker Endeward, Norbert Maassen, Dimitrios Tsikas
The most widely recognized activity of the large family of the metalloenzyme carbonic anhydrases (CAs) is the diffusion-controlled hydration of CO2 to HCO3 - and one proton, and the less rapid dehydration of HCO3 - to CO2 : CO2  + H2 O ⇆ HCO3 -  + H+ . CAs also catalyze the reaction of water with other electrophiles such as aromatic esters, sulfates and phosphates, thus contributing to lending to CAs esterase, sulfatase and phosphatase activity, respectively. Renal CAII and CAIV are involved in the reabsorption of nitrite, the autoxidation product of the signalling molecule nitric oxide (● NO): 4 ● NO + O2  + 2 H2 O → 4 ONO-  + 4 H+ ...
May 2, 2018: Analytical Biochemistry
https://www.readbyqxmd.com/read/29722897/mucopolysaccharidosis-vi-and-effects-on-growth-of-the-apical-bases-a-case-report
#3
Moema Ferreira Dos Reis, Lucas Rodrigues Pinheiro, Maria das Graças Rodrigues Pinheiro, Haroldo Amorim de Almeida, Patrícia do Socorro Queiroz Feio, Sâmia Cordovil de Almeida, Isabel Cristina Neves de Souza, Roberto Giugliani, Ida Vanessa Doederlein Schwartz, Rosely Maria Dos Santos Cavaleiro, João de Jesus Viana Pinheiro, Luiz Carlos Santana da Silva
OBJECTIVE: Mucopolysaccharidosis (MPS) VI is a rare disorder caused by an autosomal recessive mutation in the short arm of chromosome 5 (5q12-13) leading to an N-acetylgalactosamine-sulfatase lysosomal enzyme deficiency and numerous systemic clinical changes. The oral and maxillofacial complex may exhibit tooth eruption anomalies, macroglossia, gingival hypertrophy, mouth breathing, increased lower facial height, open bite, retrognathia, and progressive TMJ arthrosis. This report describes craniofacial growth changes in two MPS VI patients, sisters and daughters of outbred parents, who were longitudinally monitored from 11 to 15 years of age...
May 3, 2018: Special Care in Dentistry
https://www.readbyqxmd.com/read/29720512/a-role-for-steroid-sulfatase-in-intracrine-regulation-of-endometrial-decidualisation
#4
Douglas A Gibson, Paul A Foster, Ioannis Simitsidellis, Hilary Critchley, Olympia Kelepouri, Frances Collins, Philippa T Saunders
In women, establishment of pregnancy is dependent upon 'fine-tuning' of the endometrial microenvironment which is mediated by terminal differentiation (decidualisation) of endometrial stromal fibroblasts (ESF). We have demonstrated that intracrine steroid metabolism plays a key role in regulating decidualisation and is essential for time-dependent expression of key factors required for endometrial receptivity. The primary aim of the current study was to determine whether sulfated steroids can act as precursors to bioactive sex steroids during decidualisation...
May 2, 2018: Journal of Molecular Endocrinology
https://www.readbyqxmd.com/read/29703589/increased-gpnmb-phospho-erk1-2-and-mmp-9-in-cystic-fibrosis-in-association-with-reduced-arylsulfatase-b
#5
Sumit Bhattacharyya, Leo Feferman, Girish Sharma, Joanne K Tobacman
BACKGROUND: GPNMB was increased in a CF gene array and in Arylsulfatase B (ARSB; N-acetylgalactosamine-4-sulfatase)-null mice, consistent with previous reports that ARSB is reduced in cystic fibrosis (CF). Implications of GPNMB increase in CF are unknown. METHODS: GPNMB levels were determined in serum and circulating leukocytes from CF patients and healthy controls. GPNMB binding with β-1 integrin and measurements of phospho-ERK1/2 and MMP-9 in CFTR-uncorrected, CFTR-corrected, and normal human bronchial epithelial cells (BEC) were determined, following ARSB and GPNMB knockdown, and treatment with RGD peptide, and ERK phosphorylation inhibitor...
February 20, 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29684635/sulfatase-1-mediates-the-attenuation-of-ang-ii-induced-hypertensive-effects-by-ccl5-in-vascular-smooth-muscle-cells-from-spontaneously-hypertensive-rats
#6
Hye Ju Cha, Hye Young Kim, Hee Sun Kim
Extracellular sulfatases, sulfatase 1 (Sulf1) and sulfatase 2 (Sulf2), play a pivotal role in cell signaling and carcinogenesis. Chemokine CCL5 inhibits Ang II-induced hypertensive mediators via angiotensin II (Ang II) type 2 receptor (AT2 R) pathway in vascular smooth muscle cells (VSMCs) from spontaneously hypertensive rats (SHR). In this study, we investigated the effect of Sulfs on anti-hypertensive effects of CCL5 in SHR VSMCs. CCL5 attenuated Ang II-induced inhibition of sulfatase activity in SHR VSMCs...
April 20, 2018: Cytokine
https://www.readbyqxmd.com/read/29684479/impairment-of-the-gn%C3%AE-11-controlled-expression-of-claudin-1-and-mmp-9-and-collective-migration-of-human-breast-cancer-mcf-7-cells-by-dheas
#7
Neha Upmanyu, Ahmed Bulldan, Dimitrios Papadopoulos, Raimund Dietze, Viveka Nand Malviya, Georgios Scheiner-Bobis
Although dehydroepiandrosterone sulfate (DHEAS) constitutes the most abundant steroid in humans, in-depth investigations of its effects are rather scarce. We address here DHEAS effects on the estrogen receptor-positive metastatic human breast cancer cell line MCF-7. We focus on DHEAS-mediated signaling that might influence expression of claudin-1 and matrix metalloproteinase-9 (MMP-9), both known to be critical factors for migration and invasiveness of various cancers, including breast cancer cells. Physiological concentrations of DHEAS trigger persistent phosphorylation of Erk1/2 in MCF-7 cells...
April 20, 2018: Journal of Steroid Biochemistry and Molecular Biology
https://www.readbyqxmd.com/read/29681469/the-molecular-basis-of-polysaccharide-sulfatase-activity-and-a-nomenclature-for-catalytic-subsites-in-this-class-of-enzyme
#8
Andrew G Hettle, Chelsea Vickers, Craig S Robb, Feng Liu, Stephen G Withers, Jan-Hendrik Hehemann, Alisdair B Boraston
Sulfatases play a biologically important role by cleaving sulfate groups from molecules. They can be identified on the basis of signature sequences within their primary structures, and the largest family, S1, has predictable features that contribute specifically to the recognition and catalytic removal of sulfate groups. However, despite advances in the prediction and understanding of S1 sulfatases, a major question regards the molecular determinants that drive substrate recognition beyond the targeted sulfate group...
April 12, 2018: Structure
https://www.readbyqxmd.com/read/29678582/reversion-of-aging-related-dheas-decline-in-mouse-plasma-alleviates-aging-related-glucose-tolerance-impairment-by-potentiation-of-glucose-stimulated-insulin-secretion-of-acute-phase
#9
Junlei Ma, Jiang Yue, Rong Huang, Yu Liao, Shengxian Li, Wei Liu
AIMS/HYPOTHESIS: The latest research proposes mild age-related diabetes (MARD) as a subgroup of type 2 diabetes. While in human circulating dehydroepiandrosterone sulfate (DHEAS) decline with age is related to MARD, the role of circulating DHEAS in insulin secretion remains little known. METHODS: After intraperitoneal administration of glucose (2 g/kg) together with DHEAS (50 μg/kg) or equivalent DMSO to young (6-8 week old) or aging (12 month old) male C57BL/6 mice, plasma DHEAS and blood glucose were measured at indicated time point...
April 17, 2018: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29673857/crystalline-silica-alters-sulfatase-1-expression-in-rat-lungs-which-influences-hyper-proliferative-and-fibrogenic-effects-in-human-lung-epithelial-cells
#10
Timothy N Perkins, Paul M Peeters, Catrin Albrecht, Roel P F Schins, Mieke A Dentener, Brooke T Mossman, Emiel F M Wouters, Niki L Reynaert
Lung epithelial cells are the first cell-type to come in contact with hazardous dust materials. Upon deposition, they invoke complex reactions in attempt to eradicate particles from the airways, and repair damage. The cell surface is composed of a heterogeneous network of matrix proteins and proteoglycans, which act as scaffold and control cell-signaling networks. These functions are controlled, in part, by the sulfation patterns of heparin-sulfate proteoglycans (HSPGs), which are enzymatically regulated. Although there is evidence of altered HSPG-sulfation in idiopathic pulmonary fibrosis (IPF), this is not investigated in silicosis...
April 16, 2018: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/29672931/x-linked-ichthyosis-clinical-and-molecular-findings-in-35-italian-patients
#11
Andrea Diociaiuti, Adriano Angioni, Elisa Pisaneschi, Viola Alesi, Giovanna Zambruno, Antonio Novelli, May El Hachem
Recessive X-linked ichthyosis (XLI), the second most common ichthyosis, is caused by mutations in the STS gene encoding the steroid sulfatase enzyme. A complete deletion of the STS gene is found in 85-90% of cases. Rarely, larger deletions involving contiguous genes are detected in syndromic patients. We report the clinical and molecular genetic findings in a series of 35 consecutive Italian male patients. All patients underwent molecular testing by MLPA or aCGH, followed, in case of negative results, by next generation sequencing analysis...
April 19, 2018: Experimental Dermatology
https://www.readbyqxmd.com/read/29671225/effectiveness-of-early-hematopoietic-stem-cell-transplantation-in-preventing-neurocognitive-decline-in-mucopolysaccharidosis-type-ii-a-case-series
#12
A Selvanathan, C Ellaway, C Wilson, P Owens, P J Shaw, K Bhattacharya
The early progressive form of the X-linked disorder, Hunter syndrome or mucopolysaccharidosis type II (MPS II) (OMIM #309900), is characterized by cognitive decline, and pulmonary and cardiac complications that often cause death before 20 years of age. Deficiency of the lysosomal enzyme, iduronate-2-sulfatase (EC 3.1.6.13) results in deposition of the glycosaminoglycans, dermatan, and heparan sulfate in various tissues. In recent years, enzyme replacement therapy (ERT) has become the mainstay of treatment, but is expensive and ineffective in arresting cognitive decline...
April 19, 2018: JIMD Reports
https://www.readbyqxmd.com/read/29669302/cytotoxic-and-partial-hepatoprotective-activity-of-sodium-ascorbate-against-hepatocellular-carcinoma-through-inhibition-of-sulfatase-2-in-vivo-and-in-vitro
#13
Abdullah Alyoussef, Mohammed M H Al-Gayyar
Hepatocellular carcinoma (HCC) is characterized by elevation in the activity of sulfatase-2, an extracellular enzyme that catalyzes removal of 6-O-sulfate groups from heparan sulfate. Therefore, we conducted this study to investigate the cytotoxic activity of the strong water-soluble antioxidant, sodium ascorbate, against HCC both in vivo and in vitro. Sodium ascorbate enhanced animal survival in vivo and reduced HepG2 cells survival. The protein levels of heparan sulfate proteoglycans (HSPGs), insulin like growth factor (IGF)-2, sulfatase-2 and glypican-3 were assessed...
April 15, 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29663798/avoiding-antibiotic-inactivation-in-mycobacterium-tuberculosis-by-rv3406-through-strategic-nucleoside-modification
#14
Matthew R Bockman, Curtis A Engelhart, Surendra Dawadi, Peter Larson, Divya Tiwari, David M Ferguson, Dirk Schnappinger, Courtney C Aldrich
5'-[ N-(d-biotinoyl)sulfamoyl]amino-5'-deoxyadenosine (Bio-AMS, 1) possesses selective activity against Mycobacterium tuberculosis ( Mtb) and arrests fatty acid and lipid biosynthesis through inhibition of the Mycobacterium tuberculosis biotin protein ligase ( MtBPL). Mtb develops spontaneous resistance to 1 with a frequency of at least 1 × 10-7 by overexpression of Rv3406, a type II sulfatase that enzymatically inactivates 1. In an effort to circumvent this resistance mechanism, we describe herein strategic modification of the nucleoside at the 5'-position to prevent enzymatic inactivation...
April 17, 2018: ACS Infectious Diseases
https://www.readbyqxmd.com/read/29650477/determination-of-24-personal-care-products-in-fish-bile-using-hybrid-solvent-precipitation-and-dispersive-solid-phase-extraction-cleanup-with-ultrahigh-performance-liquid-chromatography-tandem-mass-spectrometry-and-gas-chromatography-mass-spectrometry
#15
Li Yao, Yin-Zhi Lv, Li-Juan Zhang, Wang-Rong Liu, Jian-Liang Zhao, You-Sheng Liu, Qian-Qian Zhang, Guang-Guo Ying
Personal care products (PCPs) are ubiquitous in aquatic environments owing to the continuous discharge of domestic wastewater from highly urbanized regions. These PCPs can be adsorbed by fish and thereafter usually enter the bile of the fish through biliary excretion. In this study, a sensitive method based on a combination of hybrid solvent precipitation and dispersive solid phase extraction (d-SPE) purification was developed to simultaneously extract and detect 24 PCPs, namely, 16 biocides, 4 synthetic musks, and 4 benzotriazoles, from fish bile...
April 5, 2018: Journal of Chromatography. A
https://www.readbyqxmd.com/read/29648648/fgf-signaling-deregulation-is-associated-with-early-developmental-skeletal-defects-in-animal-models-for-mucopolysaccharidosis-type-ii-mpsii
#16
Stefania Bellesso, Marika Salvalaio, Susanna Lualdi, Elisa Tognon, Roberto Costa, Paola Braghetta, Chiara Giraudo, Roberto Stramare, Laura Rigon, Mirella Filocamo, Rosella Tomanin, Enrico Moro
Skeletal abnormalities represent a major clinical burden in patients affected by the lysosomal storage disorder mucopolysaccharidosis type II (MPSII, OMIM #309900). While extensive research has emphasized the detrimental role of stored glycosaminoglycans (GAGs) in the bone marrow (BM), a limited understanding of primary cellular mechanisms underlying bone defects in MPSII has hampered the development of bone-targeted therapeutic strategies beyond enzyme replacement therapy (ERT). We here investigated the involvement of key signaling pathways related to the loss of iduronate-2-sulfatase activity in two different MPSII animal models, D...
April 10, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29618488/steroid-sulfatase-inhibition-by-aryl-sulfamates-clinical-progress-mechanism-and-future-prospects
#17
Barry V L Potter
Steroid sulfatase is an emerging drug target for the endocrine therapy of hormone-dependent diseases, catalyzing estrogen sulfate hydrolysis to estrogen. Drug discovery, developing the core aryl O-sulfamate pharmacophore, has led to steroidal and non-steroidal drugs entering numerous clinical trials, with promising results in oncology and women's health. Steroidal estrogen sulfamate derivatives were the first irreversible active-site-directed inhibitors and one was developed clinically as an oral estradiol pro-drug and for endometriosis applications...
April 4, 2018: Journal of Molecular Endocrinology
https://www.readbyqxmd.com/read/29618310/genetics-and-gene-therapy-in-hunter-disease
#18
Sestito Simona, Francesca Falvo, Rosalbina Apa, Licia Pensabene, Giuseppe Bonapace, Maria Teresa Moricca, Daniela Concolino
Mucopolysaccharidosis type II or Hunter syndrome is a rare X-linked lysosomal storage disorder due to a mutation in the gene encoding the lysosomal enzyme iduronate-2-sulfatase. The consequent enzyme deficiency leads to a progressive, multisystem accumulation of glycosaminoglycans throughout the body, which is the cause of the clinical manifestations involving also Central Nervous System for patients with the severe form of disease. The limits of the current available therapies for Hunter syndrome, hematopoietic stem cell transplantation and recombinant enzyme replacement therapy, mainly regarding brain achievement, encouraged several studies which recognized gene therapy as a potential therapeutic option for this condition...
April 4, 2018: Current Gene Therapy
https://www.readbyqxmd.com/read/29606503/a-blood-brain-barrier-penetrating-anti-human-transferrin-receptor-antibody-fusion-protein-for-neuronopathic-mucopolysaccharidosis-ii
#19
Hiroyuki Sonoda, Hideto Morimoto, Eiji Yoden, Yuri Koshimura, Masafumi Kinoshita, Galina Golovina, Haruna Takagi, Ryuji Yamamoto, Kohtaro Minami, Akira Mizoguchi, Katsuhiko Tachibana, Tohru Hirato, Kenichi Takahashi
Mucopolysaccharidosis II (MPS II) is an X-linked recessive lysosomal storage disease caused by mutations in the iduronate-2-sulfatase (IDS) gene. Since IDS catalyzes the degradation of glycosaminoglycans (GAGs), deficiency in this enzyme leads to accumulation of GAGs in most cells in all tissues and organs, resulting in severe somatic and neurological disorders. Although enzyme replacement therapy with human IDS (hIDS) has been used for the treatment of MPS II, this therapy is not effective for defects in the CNS mainly because the enzyme cannot cross the blood-brain barrier (BBB)...
March 6, 2018: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/29580682/dose-dependent-prevention-of-metabolic-and-neurologic-disease-in-murine-mps-ii-by-zfn-mediated-in-vivo-genome-editing
#20
Kanut Laoharawee, Russell C DeKelver, Kelly M Podetz-Pedersen, Michelle Rohde, Scott Sproul, Hoang-Oanh Nguyen, Tam Nguyen, Susan J St Martin, Li Ou, Susan Tom, Robert Radeke, Kathleen E Meyer, Michael C Holmes, Chester B Whitley, Thomas Wechsler, R Scott McIvor
Mucopolysaccharidosis type II (MPS II) is an X-linked recessive lysosomal disorder caused by deficiency of iduronate 2-sulfatase (IDS), leading to accumulation of glycosaminoglycans (GAGs) in tissues of affected individuals, progressive disease, and shortened lifespan. Currently available enzyme replacement therapy (ERT) requires lifelong infusions and does not provide neurologic benefit. We utilized a zinc finger nuclease (ZFN)-targeting system to mediate genome editing for insertion of the human IDS (hIDS) coding sequence into a "safe harbor" site, intron 1 of the albumin locus in hepatocytes of an MPS II mouse model...
March 10, 2018: Molecular Therapy: the Journal of the American Society of Gene Therapy
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