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https://www.readbyqxmd.com/read/28921412/how-close-are-we-to-therapies-for-sanfilippo-disease
#1
REVIEW
Lidia Gaffke, Karolina Pierzynowska, Ewa Piotrowska, Grzegorz Węgrzyn
Sanfilippo disease is one of mucopolysaccharidoses (MPS), a group of lysosomal storage diseases characterized by accumulation of partially degraded glycosaminoglycans (GAGs). It is classified as MPS type III, though it is caused by four different genetic defects, determining subtypes A, B, C and D. In each subtype of MPS III, the primary storage GAG is heparan sulfate (HS), but mutations leading to A, B, C, and D subtypes are located in genes coding for heparan N-sulfatase (the SGSH gene), α-N-acetylglucosaminidase (the NAGLU gene), acetyl-CoA:α-glucosaminide acetyltransferase (the HGSNAT gene), and N-acetylglucosamine-6-sulfatase (the GNS gene), respectively...
September 18, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28914427/the-clinical-and-genetic-spectrum-of-maroteaux-lamy-syndrome-mucopolysaccharidosis-vi-in-the-eastern-province-of-saudi-arabia
#2
Nouriya Abbas Al-Sannaa, Hind Yousif Al-Abdulwahed, Sami Ibrahim Al-Majed, Issam Hassan Bouholaigah
Mucopolysaccharidosis (MPS VI) or Maroteaux-Lamy syndrome is an autosomal recessive lysosomal storage disease caused by deficiency of the enzyme N-acetylgalactosamine 4-sulfatase or arylsulfatase B. It is involved in the degradation of glycosaminoglycans and characterized by a wide spectrum of clinical and genetic heterogeneity. So far, more than 150 mutations have been reported in the ARSB gene. Most of these mutations are either novel, private, or compound heterozygous making phenotype-genotype correlation as well as population screening difficult...
September 15, 2017: Journal of Community Genetics
https://www.readbyqxmd.com/read/28904929/diagnosing-mucopolysaccharidosis-type-iv-a-by-the-fluorometric-assay-of-n-acetylgalactosamine-6-sulfate-sulfatase-activity
#3
Sedigheh Shams, Maliheh Barazandeh Tehrani, Gabriel Civallero, Koosha Minookherad, Roberto Giugliani, Aria Setoodeh, Mohammad Taghi Haghi Ashtiani
BACKGROUND: Mucopolysaccharidosis type IVA, also known as Morquio A or MPS IV A, is an autosomal recessive disease caused by the deficiency of the lysosomal enzyme N-acetylgalactosamine-6-sulfate sulfatase (GALNS). The loss of GALNS activity leads to the impaired breakdown of glycosaminoglycans (GAGs) keratan sulfate and chondroitin-6-sulfate. The accumulation of GAGs results in multiple organ damage. The accurate and early diagnosis of this disorder helps enhance the effectiveness of the treatment...
2017: Journal of Diabetes and Metabolic Disorders
https://www.readbyqxmd.com/read/28893621/rare-genetic-variants-in-the-sodium-dependent-organic-anion-transporter-soat-slc10a6-effects-on-transport-function-and-membrane-expression
#4
Josefine Bennien, Thomas Fischer, Joachim Geyer
Sulfo-conjugated steroid hormones, such as dehydroepiandrosterone sulfate (DHEAS), pregnenolone sulfate or estrone-3-sulfate are abundant in the body, but are biologically inactive at classical androgen and estrogen steroid receptors. However, after carrier-mediated import and de-conjugation by the steroid sulfatase, these compounds participate in the overall steroid regulation of reproductive organs. The sodium-dependent organic anion transporter SOAT, coded by the SLC10A6 gene, is specific for the transport of steroid sulfates and is highly expressed in testicular germ cells, including pachytene spermatocytes, secondary spermatocytes, and round spermatids...
September 8, 2017: Journal of Steroid Biochemistry and Molecular Biology
https://www.readbyqxmd.com/read/28884488/uniqueness-of-entamoeba-sulfur-metabolism-sulfolipid-metabolism-that-plays-pleiotropic-roles-in-the-parasitic-life-cycle
#5
Fumika Mi-Ichi, Tomofumi Miyamoto, Hiroki Yoshida
Sulfur metabolism is ubiquitous and terminally synthesizes various biomolecules that are crucial for organisms, such as sulfur-containing amino acids and co-factors, sulfolipids, and sulfated saccharides. Entamoeba histolytica, a protozoan parasite responsible for amoebiasis, possesses the unique sulfur metabolism features of atypical localization and its terminal product being limited to sulfolipids. Here, we present an overall scheme of E. histolytica sulfur metabolism by relating all sulfotransferases and sulfatases to their substrates and products...
September 7, 2017: Molecular Microbiology
https://www.readbyqxmd.com/read/28882567/anaerobic-sulfatase-maturase-aslb-from-escherichia-coli-activates-human-recombinant-iduronate-2-sulfate-sulfatase-ids-and-n-acetylgalactosamine-6-sulfate-sulfatase-galns
#6
Carlos Javier Alméciga-Díaz, Andrés Dario Tolosa-Díaz, Luisa Natalia Pimentel, Yahir Andres Bonilla, Alexander Rodríguez-López, Angela J Espejo-Mojica, Juan D Patiño, Oscar F Sánchez, Janneth Gonzalez-Santos
Maturation of type I sulfatases requires the conversion of the cysteine (Cys) or serine (Ser) present in the active site to formylglycine (FGly). This activation represents a limiting step during the production of recombinant sulfatases in bacteria and eukaryotic hosts. AslB, YdeM and YidF have been proposed to participate in the activation of sulfatases in Escherichia coli. In this study, we combined in-silico and experimental approaches to study the interaction between Escherichia coli BL21(DE3) AslB and human sulfatases, more specifically iduronate-2-sulfate sulfatase (IDS) and N-acetylgalactosamine-6-sulfate sulfatase (GALNS)...
September 4, 2017: Gene
https://www.readbyqxmd.com/read/28860717/development-of-idursulfase-therapy-for-mucopolysaccharidosis-type-ii-hunter-syndrome-the-past-the-present-and-the-future
#7
REVIEW
David Ah Whiteman, Alan Kimura
Mucopolysaccharidosis type II (MPS II; Hunter syndrome; OMIM 309900) is a rare, multisystemic, progressive lysosomal storage disease caused by deficient activity of the iduronate-2-sulfatase (I2S) enzyme. Accumulation of the glycosaminoglycans dermatan sulfate and heparan sulfate results in a broad range of disease manifestations that are highly variable in presentation and severity; notably, approximately two-thirds of individuals are affected by progressive central nervous system involvement. Historically, management of this disease was palliative; however, during the 1990s, I2S was purified to homogeneity for the first time, leading to cloning of the corresponding gene and offering a means of addressing the underlying cause of MPS II using enzyme replacement therapy (ERT)...
2017: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/28857510/cell-lines-from-diamondback-moth-exhibiting-differential-susceptibility-to-baculovirus-infection-and-expressing-midgut-genes
#8
Xiao-Li Ma, Wei-Yi He, Ping Wang, Min-Sheng You
Six new cell lines were established from embryonic tissues of the diamondback moth, Plutella xylostella (L.). The cell lines showed differential characteristics, including growth in attachment or in suspension, susceptibility to a baculovirus infection and expression of genes involved in the glucosinolate detoxication pathway in P. xylostella larvae. Five of the cells lines grew attached to the culture flask and one cell line grew unattached as a suspension cell line. The cell lines had population doubling times ranging from 18 to 23 h...
August 31, 2017: Insect Science
https://www.readbyqxmd.com/read/28795252/ipet-study-an-flt-pet-window-study-to-assess-the-activity-of-the-steroid-sulfatase-inhibitor-irosustat-in-early-breast-cancer
#9
Carlo Palmieri, Richard Szydlo, Marie Miller, Laura Barker, Neva H Patel, Hironobu Sasano, Tara Barwick, Henry Tam, Dimitri Hadjiminas, Jasmin Lee, Abeer Shaaban, Hanna Nicholas, R Charles Coombes, Laura M Kenny
BACKGROUND: Steroid sulfatase (STS) is involved in oestrogen biosynthesis and irosustat is a first generation, irreversible steroid sulfatase inhibitor. A pre-surgical window-of-opportunity study with irosustat was undertaken in estrogen receptor-positive (ER+) breast cancer to assess the effect of irosustat on tumour cell proliferation as measured by 3'-deoxy-3'-[18F] fluorothymidine uptake measured by PET scanning (FLT-PET) and Ki67. METHODS: Postmenopausal women with untreated ER+ early breast cancer were recruited, and imaged with FLT-PET at baseline and after at least 2 weeks treatment with irosustat, 40 mg once daily orally...
August 9, 2017: Breast Cancer Research and Treatment
https://www.readbyqxmd.com/read/28783562/simultaneous-determination-of-paeonilactone-a-and-paeonilactone-b-in-rat-plasma-after-oral-administration-of-albiflorin-by-uplc-tof-ms-following-picolinoyl-derivatization
#10
Zhigang Wang, Shuhan Tang, Masao Hattori, Hailong Zhang, Xiuhong Wu
A new highly sensitive analytical method was developed to investigate the in vivo metabolism of albiflorin, one of the most principal components in traditional Chinese medicine. After hydrolyzation with sulfatase, the main metabolites paeonilactone A and paeonilactone B of paeoniflorin in rat plasma were successfully detected for the first time by liquid chromatography mass spectrometry following picolinoyl derivatization. Borneol was used as the internal standard compound to quantify paeonilactone A and paeonilactone B in rat plasma...
September 1, 2017: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/28743544/sodium-dependent-organic-anion-transporter-slc10a6-knockout-mice-show-normal-spermatogenesis-and-reproduction-but-elevated-serum-levels-for-cholesterol-sulfate
#11
Katharina Bakhaus, Josefine Bennien, Daniela Fietz, Alberto Sánchez-Guijo, Michaela Hartmann, Rosanna Serafini, Charles C Love, Andrei Golovko, Stefan A Wudy, Martin Bergmann, Joachim Geyer
The sodium-dependent organic anion transporter SOAT (gene name SLC10A6 in man and Slc10a6 in mice) is a plasma membrane transporter for sulfated steroids, which is highly expressed in germ cells of the testis. SOAT can transport biologically inactive sulfated steroids into specific target cells, where they can be reactivated by the steroid sulfatase (STS) to biologically active, unconjugated steroids known to regulate spermatogenesis. Significantly reduced SOAT mRNA expression was previously found in different forms of impaired spermatogenesis in man...
July 22, 2017: Journal of Steroid Biochemistry and Molecular Biology
https://www.readbyqxmd.com/read/28724898/improvement-in-the-production-of-the-human-recombinant-enzyme-n-acetylgalactosamine-6-sulfatase-rhgalns-in-escherichia-coli-using-synthetic-biology-approaches
#12
Luis H Reyes, Carolina Cardona, Luisa Pimentel, Alexander Rodríguez-López, Carlos J Alméciga-Díaz
Previously, we demonstrated production of an active recombinant human N-acetylgalactosamine-6-sulfatase (rhGALNS) enzyme in Escherichia coli as a potential therapeutic alternative for mucopolysaccharidosis IVA. However, most of the rhGALNS produced was present as protein aggregates. Here, several methods were investigated to improve production and activity of rhGALNS. These methods involved the use of physiologically-regulated promoters and alternatives to improve protein folding including global stress responses (osmotic shock), overexpression of native chaperones, and enhancement of cytoplasmic disulfide bond formation...
July 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28720494/the-eukaryotic-enzyme-bds1-is-an-alkyl-but-not-an-aryl-sulfohydrolase
#13
Grace L Waddell, Caroline R Gilmer, Nicholas G Taylor, John Randolf S Reveral, Marcello Forconi, Jennifer L Fox
The eukaryotic enzyme Bds1 in Saccharomyces cerevisiae is a metallo-β-lactamase-related enzyme evolutionarily originating from bacterial horizontal gene transfer that serves an unknown biological role. Previously, Bds1 was reported to be an alkyl and aryl sulfatase. However, we demonstrate here that Bds1 acts on primary alkyl sulfates (of 6-12 carbon atoms) but not the aryl sulfates p-nitrophenyl sulfate and p-nitrocatechol sulfate. The apparent catalytic rate constant for hydrolysis of the substrate 1-hexyl sulfate by Bds1 is over 100 times lower than that of the reaction catalyzed by its bacterial homolog SdsA1...
September 16, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28711911/human-steroid-sulfatase-induces-wnt-%C3%AE-catenin-signaling-and-epithelial-mesenchymal-transition-by-upregulating-twist1-and-hif-1%C3%AE-in-human-prostate-and-cervical-cancer-cells
#14
Sangyun Shin, Hee-Jung Im, Yeo-Jung Kwon, Dong-Jin Ye, Hyoung-Seok Baek, Donghak Kim, Hyung-Kyoon Choi, Young-Jin Chun
Steroid sulfatase (STS) catalyzes the hydrolysis of estrone sulfate and dehydroepiandrosterone sulfate (DHEAS) to their unconjugated biologically active forms. Although STS is considered a therapeutic target for estrogen-dependent diseases, the cellular functions of STS remain unclear. We found that STS induces Wnt/β-catenin s Delete ignaling in PC-3 and HeLa cells. STS increases levels of β-catenin, phospho-β-catenin, and phospho-GSK3β. Enhanced translocation of β-catenin to the nucleus by STS might activate transcription of target genes such as cyclin D1, c-myc, and MMP-7...
June 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/28710970/validation-of-a-probe-for-assessing-deconjugation-of-glucuronide-and-sulfate-phase-ii-metabolites-assayed-through-lc-ms-ms-in-biological-matrices
#15
Claire Grignon, Antoine Dupuis, Marion Albouy-Llaty, Maxime Condylis, Laurence Barrier, Pascal Carato, Bertrand Brunet, Virginie Migeot, Nicolas Venisse
LC-MS/MS has been proposed in various areas such as Therapeutic Drug Monitoring (TDM), Human Biomonitoring (HBM), disease diagnosis, clinical toxicology and doping control to identify and quantify chemical parents and their metabolites in biological matrices. To determine the total content of a xenobiotic (unconjugated+conjugated forms), an enzymatic hydrolysis step is required. Most studies in the literature have not controlled the effectiveness of the deconjugation process because no method has been described for that purpose...
September 1, 2017: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/28710204/bio-plex-immunoassay-measuring-the-quantity-of-lysosomal-n-acetylgalactosamine-6-sulfatase-protein-in-dried-blood-spots-for-the-screening-of-mucopolysaccharidosis-iva-in-newborn-a-pilot-study
#16
Chih-Kuang Chuang, Hsiang-Yu Lin, Tuan-Jen Wang, Sung-Fa Huang, Shuan-Pei Lin
OBJECTIVE: Mucopolysaccharidosis (MPS) IVA (Morquio syndrome A) is an autosomal-recessive lysosomal storage disorder caused by the deficiency of N-acetylgalactosamine-6-sulfatase (GALNS) resulting in excessive lysosomal storage of keratan sulfate. Treatments for MPS IVA have recently become available with optimal outcomes associated with early diagnosis and treatment which can be achieved by newborn screening. DESIGN: Newborn screening programme for MPS IVA pilot study...
July 13, 2017: BMJ Open
https://www.readbyqxmd.com/read/28710038/steroid-sulfatase-and-filaggrin-mutations-in-a-boy-with-severe-ichthyosis-elevated-serum-ige-level-and-moyamoya-syndrome
#17
Qian Zhang, Nuo Si, Yaping Liu, Dong Zhang, Rong Wang, Yan Zhang, Shuo Wang, Xingju Liu, Xiaofeng Deng, Yonggang Ma, Peicong Ge, Jizong Zhao, Xue Zhang
X-linked ichthyosis (XLI) is a relatively common, recessive condition caused by mutations in the steroid sulfatase (STS) gene. Common loss-of-function mutations in the filaggrin gene (FLG) cause ichthyosis vulgaris and predispose individuals to atopic eczema. We report a case of a 6-year-old boy who presented with unusually severe XLI, an increased serum immunoglobulin E level (2120IU/ml) and moyamoya angiopathy. Whole-exome sequencing identified a gross deletion encompassing the STS in Xp22.31 and the p.K4022X FLG mutation...
July 11, 2017: Gene
https://www.readbyqxmd.com/read/28697587/detection-of-bacterial-sulfatase-activity-through-liquid-and-solid-phase-colony-based-assays
#18
Hey Young Yoon, Hyung Jun Kim, Soojin Jang, Jong-In Hong
Bacterial arylsulfatases are crucial to biosynthesis in many microorganisms, as bacteria often utilize aryl sulfates as a source of sulfur. The bacterial sulfatases are associated with pathogenesis and are applied in many areas such as industry and agriculture. We developed an activity-based probe 1 for detection of bacterial sulfatase activity through liquid- and solid-phase colony-based assays. Probe 1 is hydrolyzed by sulfatase to generate fluorescent N-methyl isoindole, which is polymerized to form colored precipitates...
December 2017: AMB Express
https://www.readbyqxmd.com/read/28690541/the-significance-of-the-sulfatase-pathway-for-local-estrogen-formation-in-endometrial-cancer
#19
Maša Sinreih, Tamara Knific, Maja Anko, Neli Hevir, Katja Vouk, Aleš Jerin, Snježana Frković Grazio, Tea Lanišnik Rižner
Endometrial cancer (EC) is the most common estrogen-dependent gynecological malignancy in the developed World. To investigate the local formation of estradiol (E2), we first measured the concentrations of the steroid precursor androstenedione (A-dione) and the most potent estrogen, E2, and we evaluated the metabolism of A-dione, estrone-sulfate (E1-S), and estrone (E1) in cancerous and adjacent control endometrium. Furthermore, we studied expression of the key genes for estradiol formation via the aromatase and sulfatase pathways...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28679314/pretreatment-of-different-biological-matrices-for-exogenous-testosterone-analysis-a-review
#20
Edna Carolina Pizzato, Marcelo Filonzi, Hemerson Silva da Rosa, André Valle de Bairros
The presence of exogenous testosterone has been monitored mainly in the urine and blood. However, other biological matrices such as hair, nail, and saliva samples can be used successfully for in vivo measurement. Chromatographic analysis requires pretreatment to obtain free testosterone and its metabolites. Among the pretreatment procedures, digestion, hydrolysis and solvolysis steps are conducted to reach the analytical purpose. Digestion assay is indicated for hair and nail samples. First, it is recommended to perform the decontamination step...
July 28, 2017: Toxicology Mechanisms and Methods
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