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https://www.readbyqxmd.com/read/29209879/one-pot-analysis-of-sulfated-glycosaminoglycans
#1
C B Shrikanth, J Sanjana, Nandini D Chilkunda
Routine isolation, estimation, and characterization of glycosaminoglycans (GAGs) is quite challenging. This is compounded by the fact that the analysis is technique-intensive and more often there will be a limitation on the quantity of GAGs available for various structural, functional and biological studies. In such a scenario, the sample which can be made available for estimation and elucidation of disaccharide composition and species composition as well remains a challenge. In the present study, we have determined the feasibility where isolated sulfated GAGs (sGAG) that is estimated by metachromasia is recovered for further analysis...
December 5, 2017: Glycoconjugate Journal
https://www.readbyqxmd.com/read/29206923/vascular-abnormalities-in-the-placenta-of-chst14-fetuses-implications-in-the-pathophysiology-of-perinatal-lethality-of-the-murine-model-and-vascular-lesions-in-human-chst14-d4st1-deficiency
#2
Takahiro Yoshizawa, Shuji Mizumoto, Yuki Takahashi, Shin Shimada, Kazuyuki Sugahara, Jun Nakayama, Shin'ichi Takeda, Yoshihiro Nomura, Yuko Nitahara-Kasahara, Takashi Okada, Kiyoshi Matsumoto, Shuhei Yamada, Tomoki Kosho
Collagen is one of the most important components of the extracellular matrix that is involved in the strength of tissues, cell adhesion, and cell proliferation. Mutations in several collagen and post-translational modification enzyme genes cause Ehlers-Danlos syndrome (EDS) characterized by joint and skin hyperextensibility as well as fragility of various organs. Carbohydrate sulfotransferase 14/dermatan 4-O-sulfotransferase-1 (CHST14/D4ST1) is a critical enzyme for biosynthesis of dermatan sulfate, a side chain of various proteoglycans including biglycan that regulates collagen fibrils through their interaction...
December 1, 2017: Glycobiology
https://www.readbyqxmd.com/read/29202552/another-novel-missense-mutation-in-arsb-gene-in-iran
#3
Samaneh Abbasi, Mehrdad Noruzinia, Oranous Bashti, Mohammad Ahmadvand, Ahmad Reza Salehi Chaleshtori, Leila Mahootipou
Mucopolysaccharidosis VI (MPS-VI) is an infrequent autosomal recessive disorder caused by mutations in ARSB gene and deficiency in lysosomal enzyyme ARSB activities subsequently. This enzyme is essential for the breaking of glycosaminoglycans (GAGs) such as dermatan sulfate and chondroitin sulfate. ARSB dysfunction results in imperfect breakdown of GAGs and their accumulation in urine. Mutations in ARSB gene are the main players in MPS-VI disease and its clinical consequences. Most reported mutations are point mutations but there are some other examples in literature...
September 2017: Acta Medica Iranica
https://www.readbyqxmd.com/read/29168031/neural-cells-generated-from-human-induced-pluripotent-stem-cells-as-a-model-of-cns-involvement-in-mucopolysaccharidosis-type-ii
#4
Jitka Rybová, Jana Ledvinová, Jakub Sikora, Ladislav Kuchař, Robert Dobrovolný
Mucopolysaccharidosis type II (MPSII) is a rare X-linked lysosomal storage disorder caused by mutations in the iduronate-2-sulfatase (IDS) gene (IDS, Xq28). MPSII is characterized by skeletal deformities, hearing loss, airway obstruction, hepatosplenomegaly, cardiac valvular disease, and progressive neurological impairment. At the cellular level, IDS deficiency leads to lysosomal storage of glycosaminoglycans (GAGs), dominated by accumulation of dermatan and heparan sulfates. Human induced pluripotent stem cells (iPSC) represent an alternative system that complements the available MPSII murine model...
November 22, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29158997/presentation-and-treatments-for-mucopolysaccharidosis-type-ii-mps-ii-hunter-syndrome
#5
Molly Stapleton, Francyne Kubaski, Robert W Mason, Hiromasa Yabe, Yasuyuki Suzuki, Kenji E Orii, Tadao Orii, Shunji Tomatsu
Introduction: Mucopolysaccharidosis Type II (MPS II; Hunter syndrome) is an X- linked lysosomal storage disorder caused by a deficiency of iduronate-2-sulfatase (IDS). IDS deficiency leads to primary accumulation of dermatan sulfate (DS) and heparan sulfate (HS). MPS II is both multi-systemic and progressive. Phenotypes are classified as either attenuated or severe (based on absence or presence of central nervous system impairment, respectively). Areas covered: Current treatments available are intravenous enzyme replacement therapy (ERT), hematopoietic stem cell transplantation (HSCT), anti-inflammatory treatment, and palliative care with symptomatic surgeries...
2017: Expert Opinion on Orphan Drugs
https://www.readbyqxmd.com/read/29157190/mucopolysaccharidosis-type-vi-in-a-great-dane-caused-by-a-nonsense-mutation-in-the-arsb-gene
#6
Ping Wang, Carol Margolis, Gloria Lin, Elizabeth L Buza, Scott Quick, Karthik Raj, Rachel Han, Urs Giger
Mucopolysaccharidoses are inherited metabolic disorders that result from a deficiency of lysosomal enzymes required for the catabolism of glycosaminoglycans. Lysosomal glycosaminoglycan accumulation results in cell and organ dysfunction. This study characterized the phenotype and genotype of mucopolysaccharidosis VI in a Great Dane puppy with clinical signs of stunted growth, facial dysmorphia, skeletal deformities, corneal opacities, and increased respiratory sounds. Clinical and pathologic evaluations, urine glycosaminoglycan analyses, lysosomal enzyme assays, and ARSB sequencing were performed...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/29146595/the-cartilage-specific-lectin-c-type-lectin-domain-family-3-member-a-clec3a-enhances-tissue-plasminogen-activator-mediated-plasminogen-activation
#7
Daniela Lau, Dzemal Elezagic, Gabriele Hermes, Matthias Mörgelin, Alexander P Wohl, Manuel Koch, Ursula Hartmann, Stefan Höllriegl, Raimund Wagener, Mats Paulsson, Thomas Streichert, Andreas R Klatt
C-type lectin domain family 3 member A (CLEC3A) is a poorly characterized protein belonging to the superfamily of C-type lectins. Its closest homologue tetranectin binds to the kringle 4 domain of plasminogen and enhances its association with tissue plasminogen activator (tPA) thereby enhancing plasmin production, but whether CLEC3A contributes to plasminogen activation is unknown. Here, we recombinantly expressed murine and human full-length CLEC3As as well as truncated forms of CLEC3A in HEK-293 EBNA cells...
November 16, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29125590/novel-insights-into-antiviral-gene-regulation-of-red-swamp-crayfish-procambarus-clarkii-infected-with-white-spot-syndrome-virus
#8
Shaokui Yi, Yanhe Li, Linlin Shi, Long Zhang
White spot syndrome virus (WSSV), one of the major pathogens of Procambarus clarkii, has caused severe disruption to the aquaculture industry of P. clarkii in China. To reveal the gene regulatory mechanisms underlying WSSV infection, a comparative transcriptome analysis was performed among WSSV-infected susceptible individuals (GS), viral resistant individuals (GR), and a non-infected control group (GC). A total of 61,349 unigenes were assembled from nine libraries. Subsequently, 515 and 1033 unigenes exhibited significant differential expression in sensitive and resistant crayfish individuals compared to the control group (GC)...
November 10, 2017: Genes
https://www.readbyqxmd.com/read/29111696/sequencing-the-dermatan-sulfate-chain-of-decorin
#9
Yanlei Yu, Jiana Duan, Franklin E Leach, Toshihiko Toida, Kyohei Higashi, Hong Zhang, Fuming Zhang, I Jonathan Amster, Robert J Linhardt
Glycomics represents one of the last frontiers and most challenging in omic analysis. Glycosylation occurs in the endoplasmic reticulum and the Golgi organelle and its control is neither well-understood nor predictable based on proteomic or genomic analysis. One of the most structurally complex classes of glycoconjugates is the proteoglycans (PGs) and their glycosaminoglycan (GAG) side chains. Previously, our laboratory solved the structure of the chondroitin sulfate chain of the bikunin PG. The current study examines the much more complex structure of the dermatan sulfate GAG chain of decorin PG...
November 13, 2017: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/29081414/arylsulfatase-b-is-reduced-in-prostate-cancer-recurrences
#10
Leo Feferman, Ryan Deaton, Sumit Bhattacharyya, Hui Xie, Peter H Gann, Jonathan Melamed, Joanne K Tobacman
BACKGROUND: Arylsulfatase B (ARSB) removes the 4-sulfate group from chondroitin 4-sulfate (C4S) and dermatan sulfate and is required for their degradation. Prior work showed that ARSB immunohistochemical scores were lower in malignant prostate tissue, and were associated with higher Gleason scores and recurrence. OBJECTIVE: This study aims to confirm that ARSB immunostaining of prostate tissue obtained at the time of radical prostatectomy is prognostic for prostate cancer recurrence...
October 16, 2017: Cancer Biomarkers: Section A of Disease Markers
https://www.readbyqxmd.com/read/29063236/identification-of-differentially-expressed-genes-regulated-by-molecular-signature-in-breast-cancer-associated-fibroblasts-by-bioinformatics-analysis
#11
Basavaraj Vastrad, Chanabasayya Vastrad, Anandkumar Tengli, Sudhir Iliger
OBJECTIVE: Breast cancer is a severe risk to public health and has adequately convoluted pathogenesis. Therefore, the description of key molecular markers and pathways is of much importance for clarifying the molecular mechanism of breast cancer-associated fibroblasts initiation and progression. Breast cancer-associated fibroblasts gene expression dataset was downloaded from Gene Expression Omnibus database. METHODS: A total of nine samples, including three normal fibroblasts, three granulin-stimulated fibroblasts and three cancer-associated fibroblasts samples, were used to identify differentially expressed genes (DEGs) between normal fibroblasts, granulin-stimulated fibroblasts and cancer-associated fibroblasts samples...
October 23, 2017: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/28985469/programmed-cell-death-protein-ligand-1-silencing-with-polyethylenimine-dermatan-sulfate-complex-for-dual-inhibition-of-melanoma-growth
#12
Gijung Kwak, Dongkyu Kim, Gi-Hoon Nam, Sun Young Wang, In-San Kim, Sun Hwa Kim, Ick-Chan Kwon, Yoon Yeo
Programmed cell death protein-1 (PD-1) is a prominent immune checkpoint receptor interacting with its ligand, programmed cell death protein ligand-1 (PD-L1, B7-H1). The PD-1/PD-L1 interaction induces functional exhaustion of tumor-reactive cytotoxic T cells and, thus, interferes with antitumor T-cell immunity. In addition, PD-1/PD-L1 interaction promotes tumorigenesis via the mTOR signaling pathway in a group of cancers including melanoma. Based on the dual functions of PD-1/PD-L1 interactions in tumor progression, we hypothesize that siRNA targeting PD-L1 (siPD-L1) will suppress melanoma growth, acting on both immune checkpoint and intrinsic tumorigenesis pathways...
October 24, 2017: ACS Nano
https://www.readbyqxmd.com/read/28982054/urinary-metabolic-phenotyping-of-mucopolysaccharidosis-type-i-combining-untargeted-and-targeted-strategies-with-data-modeling
#13
Abdellah Tebani, Isabelle Schmitz-Afonso, Lenaig Abily-Donval, Bénédicte Héron, Monique Piraud, Jérôme Ausseil, Anais Brassier, Pascale De Lonlay, Farid Zerimech, Frédéric M Vaz, Bruno J Gonzalez, Stephane Marret, Carlos Afonso, Soumeya Bekri
BACKGROUND: Application of metabolic phenotyping could expand the pathophysiological knowledge of mucopolysaccharidoses (MPS) and may reveal the comprehensive metabolic impairments in MPS. However, few studies applied this approach to MPS. METHODS: We applied targeted and untargeted metabolic profiling in urine samples obtained from a French cohort comprising 19 MPS I and 15 MPS I treated patients along with 66 controls. For that purpose, we used ultra-high-performance liquid chromatography combined with ion mobility and high-resolution mass spectrometry following a protocol designed for large-scale metabolomics studies regarding robustness and reproducibility...
October 2, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28963345/sequencing-of-chondroitin-sulfate-oligosaccharides-using-a-novel-exo-lyase-from-a-marine-bacterium-that-degrades-hyaluronan-and-chondroitin-sulfate-dermatan-sulfate
#14
Wenshuang Wang, Xiaojuan Cai, Naihan Han, Wenjun Han, Kazuyuki Sugahara, Fuchuan Li
Glycosaminoglycans (GAGs) are a family of chemically heterogeneous polysaccharides that play important roles in physiological and pathological processes. Due to the structural complexity of GAGs, their sophisticated chemical structures and biological functions have not been extensively studied. Lyases that cleave GAGs are important tools for structural analysis. Although various GAG lyases have been identified, exolytic lyases with unique enzymatic property are urgently needed for GAG sequencing. In this study, a putative exolytic GAG lyase from a marine bacterium was recombinantly expressed and characterized in detail...
September 28, 2017: Biochemical Journal
https://www.readbyqxmd.com/read/28899488/investigating-the-structural-and-functional-features-of-representative-recombinants-of-chondroitinase-abc-i
#15
Khadijeh Moradi, Seyedeh Akram Shirdel, Masoumeh Shamsi, Vahab Jafarian, Khosrow Khalifeh
Chondroitin Sulfate Proteoglycans (CSPGs) are the main inhibitors for axon regeneration after damaging of Central Nervous System (CNS). Chondroitinase ABC I (cABC I) can degrade CSPGs by removing chondroitin and dermatan sulfate side chains from proteoglycans. Hence, it may be considered as an attractive candidate in biomedicine. For practical applications of this enzyme, increasing the effective circulating level and reducing the number and volume of injections for patients is one of the main concerns which is directly related to conformational stability and catalytic efficiency of the enzyme...
December 2017: Enzyme and Microbial Technology
https://www.readbyqxmd.com/read/28884960/genotypic-phenotypic-features-and-enzyme-replacement-therapy-outcome-in-patients-with-mucopolysaccharidosis-vi-from-turkey
#16
Mustafa Kılıç, Ali Dursun, Turgay Coşkun, Ayşegül Tokatlı, Rıza K Özgül, Didem Yücel-Yılmaz, Mehmet Karaca, Deniz Doğru, Dursun Alehan, Sibel Kadayıfçılar, Aydan Genç, Handan Turan-Dizdar, Burhanettin Gönüldaş, Sema Savcı, Melda Sağlam, Cemalettin Aksoy, Umut Arslan, Hatice-Serap Sivri
Mucopolysaccharidosis type VI (MPS VI) is a lysosomal storage disorder (LSD) characterized by a chronic, progressive course with multiorgan involvement. In our study, clinical, biochemical, molecular findings, and response to enzyme replacement therapy (ERT) for at least 6 months were evaluated in 20 patients with MPS VI. Treatment effects on clinical findings such as liver and spleen sizes, cardiac and respiratory parameters, visual and auditory changes, joints' range of motions, endurance tests and changes in urinary glycosaminoglycan excretions, before and after ERT were analyzed...
September 8, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28860717/development-of-idursulfase-therapy-for-mucopolysaccharidosis-type-ii-hunter-syndrome-the-past-the-present-and-the-future
#17
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/28859141/asporin-deficient-mice-have-tougher-skin-and-altered-skin-glycosaminoglycan-content-and-structure
#18
Marco Maccarana, René B Svensson, Anki Knutsson, Antonis Giannopoulos, Mea Pelkonen, MaryAnn Weis, David Eyre, Matthew Warman, Sebastian Kalamajski
The main structural component of connective tissues is fibrillar, cross-linked collagen whose fibrillogenesis can be modulated by Small Leucine-Rich Proteins/Proteoglycans (SLRPs). Not all SLRPs' effects on collagen and extracellular matrix in vivo have been elucidated; one of the less investigated SLRPs is asporin. Here we describe the successful generation of an Aspn-/- mouse model and the investigation of the Aspn-/- skin phenotype. Functionally, Aspn-/- mice had an increased skin mechanical toughness, although there were no structural changes present on histology or immunohistochemistry...
2017: PloS One
https://www.readbyqxmd.com/read/28846656/the-sea-as-a-rich-source-of-structurally-unique-glycosaminoglycans-and-mimetics
#19
REVIEW
Ariana A Vasconcelos, Vitor H Pomin
Glycosaminoglycans (GAGs) are sulfated glycans capable of regulating various biological and medical functions. Heparin, heparan sulfate, chondroitin sulfate, dermatan sulfate, keratan sulfate and hyaluronan are the principal classes of GAGs found in animals. Although GAGs are all composed of disaccharide repeating building blocks, the sulfation patterns and the composing alternating monosaccharides vary among classes. Interestingly, GAGs from marine organisms can present structures clearly distinct from terrestrial animals even considering the same class of GAG...
August 28, 2017: Microorganisms
https://www.readbyqxmd.com/read/28822024/glycosaminoglycans-and-glycolipids-as-potential-biomarkers-in-lung-cancer
#20
Guoyun Li, Lingyun Li, Eun Ji Joo, Ji Woong Son, Young Jin Kim, Jae Ku Kang, Kyung Bok Lee, Fuming Zhang, Robert J Linhardt
In this report, we used liquid chromatography-mass spectrometry and Western blotting to analyze the content and structure of glycosaminoglycans, glycolipids and selected proteins to compare differences between patient-matched normal and cancerous lung tissues obtained from lung cancer patients. The cancer tissue samples contained over twice as much chondroitin sulfate (CS)/dermatan sulfate (DS) as did the normal tissue samples, while the amount of heparan sulfate (HS) and hyaluronan (HA) in normal and cancer tissues were not significantly different...
October 2017: Glycoconjugate Journal
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