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https://www.readbyqxmd.com/read/29440708/differential-role-of-fl-bid-and-t-bid-during-verotoxin-1-induced-apoptosis-in-burkitt-s-lymphoma-cells
#1
Justine Debernardi, Emilie Hollville, Marc Lipinski, Joëlle Wiels, Aude Robert
The globotriaosylceramide Gb3 is a glycosphingolipid expressed on a subpopulation of germinal center B lymphocytes which has been recognized as the B cell differentiation antigen CD77. Among tumoral cell types, Gb3/CD77 is strongly expressed in Burkitt's lymphoma (BL) cells as well as other solid tumors including breast, testicular and ovarian carcinomas. One known ligand of Gb3/CD77 is Verotoxin-1 (VT-1), a Shiga toxin produced in specific E. coli strains. Previously, we have reported that in BL cells, VT-1 induces apoptosis via a caspase-dependent and mitochondria-dependent pathway...
February 14, 2018: Oncogene
https://www.readbyqxmd.com/read/29432805/intracellular-drug-delivery-potential-usefulness-of-engineered-shiga-toxin-subunit-b-for-targeted-cancer-therapy
#2
REVIEW
Vera Luginbuehl, Nicolas Meier, Karin Kovar, Jack Rohrer
A treasure trove of intracellular cancer drug targets remains hidden behind cell membranes. However, engineered pathogen-derived toxins such as Shiga toxins can deliver small or macromolecular drugs to specific intracellular organelles. After binding to ganglioglobotriaosylceramide (Gb3, CD77), the non-toxic subunit B (StxB) of the Shiga-holotoxin is endocytosed and delivers its payload by a unique retrograde trafficking pathway via the endoplasmic reticulum to the cytosol. This review provides an overview of biomedical applications of StxB-based drug delivery systems in targeted cancer diagnosis and therapy...
February 9, 2018: Biotechnology Advances
https://www.readbyqxmd.com/read/29422803/distribution-of-cytomegalovirus-genotypes-among-ulcerative-colitis-patients-in-okinawa-japan
#3
Saifun Nahar, Akira Hokama, Atsushi Iraha, Tetsuya Ohira, Tetsu Kinjo, Tetsuo Hirata, Takeshi Kinjo, Gretchen L Parrott, Jiro Fujita
Background/Aims: To determine the prevalence of glycoprotein B (gB), glycoprotein N (gN), and glycoprotein H (gH) genotypes of human cytomegalovirus (HCMV) superimposed on ulcerative colitis (UC) patients in Japan. Methods: Four archived stool samples and 7-archived extracted DNA from stool samples of 11 UC patients with positive multiplex polymerase chain reaction (PCR) results for HCMV were used UL55 gene encoding gB, UL73 gene encoding gN, and UL75 gene encoding gH were identified by PCR...
January 2018: Intestinal Research
https://www.readbyqxmd.com/read/29402852/cytomegalovirus-glycoprotein-b-genotype-distribution-in-italian-transplant-patients
#4
Marco Ciotti, Eleonora Cella, Massimo Rittà, Massimo Ciccozzi, Rossana Cavallo, Carlo Federico Perno, Cristina Costa
BACKGROUND: The cytomegalovirus (CMV) UL55 gene encodes for a glycoprotein implicated in virus pathogenesis. Based on UL55 polymorphism, CMV has been divided into 4 genotypes. Previous studies investigated the possible role of genotypes in the clinical outcome of infection in different categories of patients; however, few data are available, particularly in the transplant setting and Italian case records. METHODS: Phylogenetic analysis through a maximum likelihood tree was used to evaluate the prevalence and distribution of CMV genotypes in whole blood specimens from 47 transplant patients and investigate the relation with demographic and clinical features...
February 2, 2018: Intervirology
https://www.readbyqxmd.com/read/29343457/sphingolipid-signaling-in-renal-fibrosis
#5
REVIEW
Andrea Huwiler, Josef Pfeilschifter
Over the last decade, various sphingolipid subspecies have gained increasing attention as important signaling molecules that regulate a multitude of physiological and pathophysiological processes including inflammation and tissue remodeling. These mediators include ceramide, sphingosine 1-phosphate (S1P), the cerebroside glucosylceramide, lactosylceramide, and the gangliosides GM3 and Gb3. These lipids have been shown to accumulate in various chronic kidney diseases that typically end in renal fibrosis and ultimately renal failure...
January 14, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29288396/plasma-lyso-gb3-a-biomarker-for-monitoring-fabry-patients-during-enzyme-replacement-therapy
#6
Hitoshi Sakuraba, Tadayasu Togawa, Takahiro Tsukimura, Hiroshi Kato
BACKGROUND: Recently, globotriaosylsphingosine (lyso-Gb3) has attracted interest as a biomarker of Fabry disease. However, little is known regarding its utility for the evaluation of the therapeutic efficacy. METHOD: We measured plasma lyso-Gb3 concentration in Japanese healthy subjects and Fabry patients by means of liquid chromatography-tandem mass spectrometry (LC-MS/MS). We determined the reference interval in Japanese (UMIN000016854), and examined the effect of enzyme replacement therapy (ERT) with recombinant α-galactosidase A (GLA) and the influence of antibodies against the enzyme on the plasma lyso-Gb3 level in Fabry patients (UMIN000017152)...
December 29, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29274327/investigation-of-correlation-of-urinary-globotriaosylceramide-gb3-levels-with-markers-of-renal-function-in-patients-with-fabry-disease
#7
Alana Pimentel Moura, Tatiane Hammerschmidt, Marion Deon, Roberto Giugliani, Carmen Regla Vargas
Fabry disease (FD) is a disorder that results from mutations of hydrolase α-galactosidase A. The enzymatic defect leads to accumulation of globotriaosylceramide (Gb3) in the kidney. Substrate deposition is related to tissue damage in FD, but the relation of urinary Gb3 levels in patients and the renal function markers remain not completely understood. Once nephropathy is one of the main features of FD and is marked by an insidious development, we investigated a possible correlation of Gb3 with biochemical markers of nephropathy including albuminuria, estimated glomerular filtration rate (eGFR), serum creatinine and urea, and proteinuria in male and female patients under or not enzyme replacement therapy (ERT)...
December 21, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/29215092/improvement-in-the-sensitivity-of-newborn-screening-for-fabry-disease-among-females-through-the-use-of-a-high-throughput-and-cost-effective-method-dna-mass-spectrometry
#8
Yung-Hsiu Lu, Po-Hsun Huang, Li-Yun Wang, Ting-Rong Hsu, Hsing-Yuan Li, Pi-Chang Lee, Yu-Ping Hsieh, Sheng-Che Hung, Yu-Chen Wang, Sheng-Kai Chang, Ya-Ting Lee, Ping-Hsun Ho, Hui-Chen Ho, Dau-Ming Niu
Many female carriers of Fabry disease are likely to develop severe morbidity and mortality. However, by our own estimation, around 80% of female newborns are missed by our current enzyme-based screening approach. Our team's aim was to develop an improved cost-effective screening method that is able to detect Fabry disease among female newborns. In Taiwan, based on a database of 916,000 newborns, ~98% of Fabry patients carry mutations out of a pool of only 21 pathogenic mutations. An Agena iPLEX platform was designed to detect these 21 pathogenic mutations using only a single-assay panel...
November 15, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/29196726/emergence-of-ba9-genotype-of-human-respiratory-syncytial-virus-subgroup-b-in-china-from-2006-to-2014
#9
Jinhua Song, Huiling Wang, Jing Shi, Aili Cui, Yanzhi Huang, Liwei Sun, Xingyu Xiang, Chaofeng Ma, Pengbo Yu, Zifeng Yang, Qi Li, Teresa I Ng, Yan Zhang, Rongbo Zhang, Wenbo Xu
A study was conducted to investigate the circulation of HRSV subgroup B (HRSVB) in China in recent years. HRSVB sequences from 365 samples collected in 1991, 2004 and 2008-2014 in China, together with 332 Chinese HRSVB sequences obtained from GenBank were analyzed to determine the geographic and yearly distribution of HRSVB. Phylogenetic analysis revealed these HRSVB sequences clustered into 4 genotypes with different frequencies: BA (83%), CB1 (11%), SAB (3.0%) and GB3 (0.7%). Between 2005 and 2013, there was a co-circulation of BA and non-BA genotypes in China...
December 1, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29196214/the-lysosomal-enzyme-alpha-galactosidase-a-is-deficient-in-parkinson-s-disease-brain-in-association-with-the-pathologic-accumulation-of-alpha-synuclein
#10
Michael P Nelson, Michel Boutin, Tonia E Tse, Hailin Lu, Emily D Haley, Xiaosen Ouyang, Jianhua Zhang, Christiane Auray-Blais, John J Shacka
The aberrant accumulation of alpha-synuclein (α-syn) is believed to contribute to the onset and pathogenesis of Parkinson's disease (PD). The autophagy-lysosome pathway (ALP) is responsible for the high capacity clearance of α-syn. ALP dysfunction is documented in PD and pre-clinical evidence suggests that inhibiting the ALP promotes the pathological accumulation of α-syn. We previously identified the pathological accumulation of α-syn in the brains of mice deficient for the soluble lysosomal enzyme alpha-Galactosidase A (α-Gal A), a member of the glycosphingolipid metabolism pathway...
December 2, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/29186537/fabry-disease-under-enzyme-replacement-therapy-new-insights-in-efficacy-of-different-dosages
#11
Johannes Krämer, Malte Lenders, Sima Canaan-Kühl, Peter Nordbeck, Nurcan Üçeyler, Daniela Blaschke, Thomas Duning, Stefanie Reiermann, Jörg Stypmann, Stefan-Martin Brand, Timo Gottschling, Stefan Störk, Christoph Wanner, Claudia Sommer, Eva Brand, Frank Weidemann
Background: Fabry patients on reduced dose of agalsidase-beta or after switch to agalsidase-alfa show a decline in estimated glomerular filtration rate (eGFR) and an increase of the Mainz Severity Score Index. Methods: In this prospective observational study, we assessed end-organ damage and clinical symptoms in 112 patients who had received agalsidase-beta (1.0 mg/kg) for >1 year, who were (i) non-randomly assigned to continue this treatment regime (regular-dose group, n = 37); (ii) received a reduced dose of agalsidase-beta and subsequent switch to agalsidase-alfa (0...
November 23, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29161295/cardiac-and-renal-dysfunction-is-associated-with-progressive-hearing-loss-in-patients-with-fabry-disease
#12
Maria Köping, Wafaa Shehata-Dieler, Mario Cebulla, Kristen Rak, Daniel Oder, Jonas Müntze, Peter Nordbeck, Christoph Wanner, Rudolf Hagen, Sebastian Schraven
BACKGROUND: Fabry disease (FD) is an X-linked recessive hereditary lysosomal storage disorder which results in the accumulation of globotriaosylceramid (Gb3) in tissues of kidney and heart as well as central and peripheral nervous system. Besides prominent renal and cardiac organ involvement, cochlear symptoms like high-frequency hearing loss and tinnitus are frequently found with yet no comprehensive data available in the literature. OBJECTIVE: To examine hearing loss in patients with FD depending on cardiac and renal function...
2017: PloS One
https://www.readbyqxmd.com/read/29116557/bidirectional-band-selective-magnetization-transfer-along-the-protein-backbone-doubles-the-information-content-of-solid-state-nmr-correlation-experiments
#13
M M Jolly, J A Jarvis, M Carravetta, M H Levitt, P T F Williamson
Resonance assignment is the first stage towards solving the structure of a protein. This is normally achieved by the employment of separate inter and intra residue experiments. By utilising the mixed rotation and rotary recoupling (MIRROR) condition it is possible to double the information content through the efficient bidirectional transfer of magnetization from the CO to its adjacent Cα and the Cα of the subsequent amino acid. We have incorporated this into a 3D experiment, a 3D-MIRROR-NCOCA, where correlations present in the 3D spectrum permit the sequential assignment of the protein backbone from a single experiment as we have demonstrated on a microcrystalline preparation of GB3...
November 8, 2017: Journal of Biomolecular NMR
https://www.readbyqxmd.com/read/29099167/separation-and-analysis-of-lactosylceramide-galabiosylceramide-and-globotriaosylceramide-by-lc-ms-ms-in-urine-of-fabry-disease-patients
#14
Michel Boutin, Iskren Menkovic, Tristan Martineau, Vanessa Vaillancourt-Lavigueur, Amanda Toupin, Christiane Auray-Blais
Fabry disease is an X-linked lysosomal storage disorder caused by α-galactosidase A (α-GAL A) deficiency. This enzyme contributes to the cellular recycling of glycosphingolipids such as galabiosylceramide (Ga2), globotriaosylceramide (Gb3), and globotriaosylsphingosine (lyso-Gb3) by hydrolyzing the terminal α-galactosyl moiety. Urine and plasma α-GAL A substrates are currently analyzed as biomarkers for the detection, monitoring, and follow-up of Fabry disease patients. The sensitivity of the analysis of Ga2 is decreased by the co-analysis of its structural isomer, lactosylceramide (LacCer), which is not an α-GAL A substrate...
November 27, 2017: Analytical Chemistry
https://www.readbyqxmd.com/read/29096508/links-between-the-charge-model-and-bonded-parameter-force-constants-in-biomolecular-force-fields
#15
David S Cerutti, Karl T Debiec, David A Case, Lillian T Chong
The ff15ipq protein force field is a fixed charge model built by automated tools based on the two charge sets of the implicitly polarized charge method: one set (appropriate for vacuum) for deriving bonded parameters and the other (appropriate for aqueous solution) for running simulations. The duality is intended to treat water-induced electronic polarization with an understanding that fitting data for bonded parameters will come from quantum mechanical calculations in the gas phase. In this study, we compare ff15ipq to two alternatives produced with the same fitting software and a further expanded data set but following more conventional methods for tailoring bonded parameters (harmonic angle terms and torsion potentials) to the charge model...
October 28, 2017: Journal of Chemical Physics
https://www.readbyqxmd.com/read/29072716/measurement-of-14-n-quadrupole-couplings-in-biomolecular-solids-using-indirect-detection-14-n-solid-state-nmr-with-dnp
#16
J A Jarvis, I Haies, M Lelli, A J Rossini, I Kuprov, M Carravetta, P T F Williamson
The quadrupolar interaction experienced by the spin-1 (14)N nucleus is known to be extremely sensitive to local structure and dynamics. Furthermore, the (14)N isotope is 99.6% naturally abundant, making it an attractive target for characterisation of nitrogen-rich biological molecules by solid-state NMR. In this study, dynamic nuclear polarization (DNP) is used in conjunction with indirect (14)N detected solid-state NMR experiments to simultaneously characterise the quadrupolar interaction at multiple (14)N sites in the backbone of the microcrystalline protein, GB3...
November 7, 2017: Chemical Communications: Chem Comm
https://www.readbyqxmd.com/read/28988177/fabry-disease-due-to-d313y-and-novel-gla-mutations
#17
Konstantinos Koulousios, Konstantinos Stylianou, Panagiotis Pateinakis, Maria Zamanakou, Gedeon Loules, Eleni Manou, Parthena Kyriklidou, Christos Katsinas, Alexandra Ouzouni, John Kyriazis, Matthaios Speletas, Anastasios E Germenis
OBJECTIVES: Our aim is to report four novel α-gal A gene (GLA) mutations resulting in Fabry disease (FD) and provide evidence of pathogenicity of the D313Y mutation regarding which contradictory data have been presented in the literature. SETTING AND PARTICIPANTS: Twenty-five family members of nine unrelated patients with definite FD diagnosis, 10 clinically suspected cases and 18 members of their families were included in this polycentric cohort study. PRIMARY AND SECONDARY OUTCOME MEASURES: Genotyping and measurement of lyso-Gb3 was performed in all individuals...
October 6, 2017: BMJ Open
https://www.readbyqxmd.com/read/28947349/contribution-of-inflammatory-pathways-to-fabry-disease-pathogenesis
#18
REVIEW
Paula Rozenfeld, Sandro Feriozzi
Lysosomal storage diseases are usually considered to be pathologies in which the passive deposition of unwanted materials leads to functional changes in lysosomes. Lysosomal deposition of unmetabolized glycolipid substrates stimulates the activation of pathogenic cascades, including immunological processes, and particularly the activation of inflammation. In lysosomal storage diseases, the inflammatory response is continuously being activated because the stimulus cannot be eliminated. Consequently, inflammation becomes a chronic process...
September 13, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28941080/gb3-glycosphingolipids-with-fluorescent-oligoene-fatty-acids-synthesis-and-phase-behavior-in-model-membranes
#19
Lukas J Patalag, Jeremias Sibold, Ole M Schütte, Claudia Steinem, Daniel B Werz
Glycosphingolipids are involved in a number of physiological and pathophysiological processes, and they serve as receptors for a variety of bacterial toxins and viruses. To investigate their function in lipid membranes, fluorescently labeled glycosphingolipids are highly desirable. Herein, a synthetic route to access Gb3 glycosphingolipids with fluorescently labeled fatty acids, consisting of pentaene and hexaene moieties either at the terminus or in the middle of the acyl chain, has been developed. The fluorescent properties of the Gb3 derivatives were investigated in small unilamellar vesicles composed of a raft-like mixture...
September 20, 2017: Chembiochem: a European Journal of Chemical Biology
https://www.readbyqxmd.com/read/28933415/integrative-systems-biology-investigation-of-fabry-disease
#20
Marco Fernandes, Holger Husi
Fabry disease (FD) is a rare X-linked recessive genetic disorder caused by a deficient activity of the lysosomal enzyme alpha-galactosidase A (GLA) and is characterised by intra-lysosomal accumulation of globotriaosylceramide (Gb3). We performed a meta-analysis of peer-reviewed publications including high-throughput omics technologies including naïve patients and those undergoing enzyme replacement therapy (ERT). This study describes FD on a systems level using a systems biology approach, in which molecular data sourced from multi-omics studies is extracted from the literature and integrated as a whole in order to reveal the biochemical processes and molecular pathways potentially affected by the dysregulation of differentially expressed molecules...
November 15, 2016: Diseases (Basel)
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