Globotriaosylceramide (BioDeep_00000911034)
代谢物信息卡片
Globotriaosylceramide
化学式: C38H69NO18 (827.4514414)
中文名称:
谱图信息:
最多检出来源 () 0%
分子结构信息
SMILES: CCCCCCCCCCCCCC=CC(C(COC1C(C(C(C(O1)CO)OC2C(C(C(C(O2)CO)OC3C(C(C(C(O3)CO)O)O)O)O)O)O)O)NC(=O)C)O
InChI: InChI=1S/C38H69NO18/c1-3-4-5-6-7-8-9-10-11-12-13-14-15-16-23(44)22(39-21(2)43)20-52-36-32(50)29(47)34(25(18-41)54-36)57-38-33(51)30(48)35(26(19-42)55-38)56-37-31(49)28(46)27(45)24(17-40)53-37/h15-16,22-38,40-42,44-51H,3-14,17-20H2,1-2H3,(H,39,43)/b16-15+/t22-,23+,24+,25+,26+,27-,28-,29+,30+,31+,32+,33+,34+,35-,36+,37+,38-/m0/s1
相关代谢途径
Reactome(0)
BioCyc(0)
PlantCyc(0)
代谢反应
0 个相关的代谢反应过程信息。
Reactome(0)
BioCyc(0)
WikiPathways(0)
Plant Reactome(0)
INOH(0)
PlantCyc(0)
COVID-19 Disease Map(0)
PathBank(0)
PharmGKB(0)
0 个相关的物种来源信息
在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有：
- PubMed: 来源于PubMed文献库中的文献信息，我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表，这个列表按照代谢物同时出现的文献数量降序排序，取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
- NCBI Taxonomy: 通过文献数据挖掘，得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序，取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
- Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
- Chemical Reaction: 在化学反应过程中，存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。
点击图上的相关代谢物的名称，可以跳转到相关代谢物的信息页面。
文献列表
- Behzad Najafian, Aurelio Silvestroni, Alexey Sokolovskiy, Camilla Tøndel, Einar Svarstad, Bogdan Obrisca, Gener Ismail, Myrl D Holida, Michael Mauer. A novel unbiased method reveals progressive podocyte globotriaosylceramide accumulation and loss with age in females with Fabry disease.
Kidney international.
2022 Jul; 102(1):173-182. doi:
10.1016/j.kint.2022.03.023. [PMID: 35483528] - Jong Hun An, Sang-Eun Hong, Seong-Lan Yu, Jaeku Kang, Chang Gyo Park, Hoi Young Lee, Sung-Ki Lee, Dong Chul Lee, Hwan-Woo Park, Won-Min Hwang, Sung-Ro Yun, Yohan Park, Moon Hyang Park, Kuk Ro Yoon, Se-Hee Yoon. Ceria-Zirconia nanoparticles reduce intracellular globotriaosylceramide accumulation and attenuate kidney injury by enhancing the autophagy flux in cellular and animal models of Fabry disease.
Journal of nanobiotechnology.
2022 Mar; 20(1):125. doi:
10.1186/s12951-022-01318-8. [PMID: 35264192] - Alessandro Perrone, Susan Mohamed, Vincenzo Donadio, Rocco Liguori, Manuela Contin. A Rapid and Simple UHPLC-MS/MS Method for Quantification of Plasma Globotriaosylsphingosine (lyso-Gb3).
Molecules (Basel, Switzerland).
2021 Dec; 26(23):. doi:
10.3390/molecules26237358. [PMID: 34885938] - Antonino Tuttolomondo, Irene Simonetta, Renata Riolo, Federica Todaro, Tiziana Di Chiara, Salvatore Miceli, Antonio Pinto. Pathogenesis and Molecular Mechanisms of Anderson-Fabry Disease and Possible New Molecular Addressed Therapeutic Strategies.
International journal of molecular sciences.
2021 Sep; 22(18):. doi:
10.3390/ijms221810088. [PMID: 34576250] - Johanna Detzner, Charlotte Püttmann, Gottfried Pohlentz, Hans-Ulrich Humpf, Alexander Mellmann, Helge Karch, Johannes Müthing. Primary Human Colon Epithelial Cells (pHCoEpiCs) Do Express the Shiga Toxin (Stx) Receptor Glycosphingolipids Gb3Cer and Gb4Cer and Are Largely Refractory but Not Resistant towards Stx.
International journal of molecular sciences.
2021 09; 22(18):. doi:
10.3390/ijms221810002. [PMID: 34576167] - Sandro Feriozzi, Paula Rozenfeld. Pathology and pathogenic pathways in fabry nephropathy.
Clinical and experimental nephrology.
2021 Sep; 25(9):925-934. doi:
10.1007/s10157-021-02058-z. [PMID: 33768330] - Allan Wee Ren Ng, Kumaran Narayanan. An antibody binding-based fluorescent assay for the rapid quantification of globotriaosylceramide levels in human Fabry cells.
Analytical biochemistry.
2021 09; 628(?):114287. doi:
10.1016/j.ab.2021.114287. [PMID: 34119486] - Johanna Detzner, Anna-Lena Klein, Gottfried Pohlentz, Elisabeth Krojnewski, Hans-Ulrich Humpf, Alexander Mellmann, Helge Karch, Johannes Müthing. Primary Human Renal Proximal Tubular Epithelial Cells (pHRPTEpiCs): Shiga Toxin (Stx) Glycosphingolipid Receptors, Stx Susceptibility, and Interaction with Membrane Microdomains.
Toxins.
2021 07; 13(8):. doi:
10.3390/toxins13080529. [PMID: 34437399] - Anke Schumann, Kristin Schaller, Véronique Belche, Markus Cybulla, Sarah C Grünert, Nicolai Moers, Jörn O Sass, Andres Kaech, Luciana Hannibal, Ute Spiekerkoetter. Defective lysosomal storage in Fabry disease modifies mitochondrial structure, metabolism and turnover in renal epithelial cells.
Journal of inherited metabolic disease.
2021 07; 44(4):1039-1050. doi:
10.1002/jimd.12373. [PMID: 33661535] - Mouhannad Malek, Anna M Wawrzyniak, Peter Koch, Christian Lüchtenborg, Manuel Hessenberger, Timo Sachsenheimer, Wonyul Jang, Britta Brügger, Volker Haucke. Inositol triphosphate-triggered calcium release blocks lipid exchange at endoplasmic reticulum-Golgi contact sites.
Nature communications.
2021 05; 12(1):2673. doi:
10.1038/s41467-021-22882-x. [PMID: 33976123] - Annette Brandel, Sahaja Aigal, Simon Lagies, Manuel Schlimpert, Ana Valeria Meléndez, Maokai Xu, Anika Lehmann, Daniel Hummel, Daniel Fisch, Josef Madl, Thorsten Eierhoff, Bernd Kammerer, Winfried Römer. The Gb3-enriched CD59/flotillin plasma membrane domain regulates host cell invasion by Pseudomonas aeruginosa.
Cellular and molecular life sciences : CMLS.
2021 Apr; 78(7):3637-3656. doi:
10.1007/s00018-021-03766-1. [PMID: 33555391] - Yang Liu, Songhai Tian, Hatim Thaker, Min Dong. Shiga Toxins: An Update on Host Factors and Biomedical Applications.
Toxins.
2021 03; 13(3):. doi:
10.3390/toxins13030222. [PMID: 33803852] - Aneal Khan, Dwayne L Barber, Ju Huang, C Anthony Rupar, Jack W Rip, Christiane Auray-Blais, Michel Boutin, Pamela O'Hoski, Kristy Gargulak, William M McKillop, Graeme Fraser, Syed Wasim, Kaye LeMoine, Shelly Jelinski, Ahsan Chaudhry, Nicole Prokopishyn, Chantal F Morel, Stephen Couban, Peter R Duggan, Daniel H Fowler, Armand Keating, Michael L West, Ronan Foley, Jeffrey A Medin. Lentivirus-mediated gene therapy for Fabry disease.
Nature communications.
2021 02; 12(1):1178. doi:
10.1038/s41467-021-21371-5. [PMID: 33633114] - Johanna Detzner, Elisabeth Krojnewski, Gottfried Pohlentz, Daniel Steil, Hans-Ulrich Humpf, Alexander Mellmann, Helge Karch, Johannes Müthing. Shiga Toxin (Stx)-Binding Glycosphingolipids of Primary Human Renal Cortical Epithelial Cells (pHRCEpiCs) and Stx-Mediated Cytotoxicity.
Toxins.
2021 02; 13(2):. doi:
10.3390/toxins13020139. [PMID: 33673393] - Carmen Jarne, Luis Membrado, María Savirón, Jesús Vela, Jesús Orduna, Rosa Garriga, Javier Galbán, Vicente L Cebolla. Globotriaosylceramide-related biomarkers of fabry disease identified in plasma by high-performance thin-layer chromatography - densitometry- mass spectrometry.
Journal of chromatography. A.
2021 Feb; 1638(?):461895. doi:
10.1016/j.chroma.2021.461895. [PMID: 33477028] - Cosimo Andrea Stamerra, Martina De Feo, Vanessa Castelli, Michele d'Angelo, Annamaria Cimini, Davide Grassi, Claudio Ferri. Effects of agalsidase-β administration on vascular function and blood pressure in familial Anderson-Fabry disease.
European journal of human genetics : EJHG.
2021 02; 29(2):218-224. doi:
10.1038/s41431-020-00721-9. [PMID: 32948848] - Leonardo Ulivi, Baris Kanber, Ferran Prados, Indran Davagnanam, Aine Merwick, Edgar Chan, Fay Williams, Derralynn Hughes, Elaine Murphy, R H Lachmann, Claudia A M Gandini Wheeler-Kingshott, Lisa Cipolotti, David J Werring. White matter integrity correlates with cognition and disease severity in Fabry disease.
Brain : a journal of neurology.
2020 12; 143(11):3331-3342. doi:
10.1093/brain/awaa282. [PMID: 33141169] - Yüksel Çavuşoğlu, Ebru Özpelit, Nur Arslan, Mesut Demir, Gökhan Kahveci, Hüseyin Onay, Emir Özgür Barış Ökçün, Omaç Tufekcioglu, Selcen Yakar Tülüce, Gonca Kılıç Yıldırım. [Fabry disease in cardiology: Diagnosis and therapeutic approaches].
Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir.
2020 12; 48(Suppl 2):1-42. doi:
10.5543/tkda.2020.38262. [PMID: 33476309] - Sam Kant, Mohamed G Atta. Therapeutic advances in Fabry disease: The future awaits.
Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie.
2020 Nov; 131(?):110779. doi:
10.1016/j.biopha.2020.110779. [PMID: 33152937] - Grigoris Effraimidis, Åse K Rasmussen, Henning Bundgaard, Søren S Sørensen, Ulla Feldt-Rasmussen. Is the alpha-galactosidase A variant p.Asp313Tyr (p.D313Y) pathogenic for Fabry disease? A systematic review.
Journal of inherited metabolic disease.
2020 09; 43(5):922-933. doi:
10.1002/jimd.12240. [PMID: 32246457] - Michel Boutin, Pamela Lavoie, Iskren Menkovic, Amanda Toupin, Mona Abaoui, Maha Elidrissi-Elawad, Marie-Françoise Arthus, Carole Fortier, Claudia Ménard, Bruno Maranda, Daniel G Bichet, Christiane Auray-Blais. Diurnal Variation of Urinary Fabry Disease Biomarkers during Enzyme Replacement Therapy Cycles.
International journal of molecular sciences.
2020 Aug; 21(17):. doi:
10.3390/ijms21176114. [PMID: 32854306] - Thomas Schubert, Taras Sych, Josef Madl, Maokai Xu, Ramin Omidvar, Lukas J Patalag, Annika Ries, Katharina Kettelhoit, Annette Brandel, Yves Mely, Claudia Steinem, Daniel B Werz, Roland Thuenauer, Winfried Römer. Differential recognition of lipid domains by two Gb3-binding lectins.
Scientific reports.
2020 06; 10(1):9752. doi:
10.1038/s41598-020-66522-8. [PMID: 32546842] - Satoshi Ishii, Atsumi Taguchi, Nozomu Okino, Makoto Ito, Hiroki Maruyama. Determination of globotriaosylceramide analogs in the organs of a mouse model of Fabry disease.
The Journal of biological chemistry.
2020 04; 295(17):5577-5587. doi:
10.1074/jbc.ra120.012665. [PMID: 32179651] - Behzad Najafian, Camilla Tøndel, Einar Svarstad, Marie-Claire Gubler, João-Paulo Oliveira, Michael Mauer. Accumulation of Globotriaosylceramide in Podocytes in Fabry Nephropathy Is Associated with Progressive Podocyte Loss.
Journal of the American Society of Nephrology : JASN.
2020 04; 31(4):865-875. doi:
10.1681/asn.2019050497. [PMID: 32127409] - Sae Aratani, Hiroyuki Yamakawa, Shinya Suzuki, Tomoyuki Otsuka, Yukinao Sakai, Akira Shimizu, Shuichi Tsuruoka. A case of female Fabry disease revealed by renal biopsy.
CEN case reports.
2020 02; 9(1):24-29. doi:
10.1007/s13730-019-00420-5. [PMID: 31542871] - Christiane Auray-Blais, Pamela Lavoie, Mona Abaoui, Anne-Marie Côté, Michel Boutin, Ayub Akbari, Adeera Levin, Fabrice Mac-Way, Joe Tr Clarke. High-risk screening for Fabry disease in a Canadian cohort of chronic kidney disease patients.
Clinica chimica acta; international journal of clinical chemistry.
2020 Feb; 501(?):234-240. doi:
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Revista da Associacao Medica Brasileira (1992).
2020 Jan; 66Suppl 1(Suppl 1):s10-s16. doi:
10.1590/1806-9282.66.s1.10. [PMID: 31939530] - Daisuke Kami, Masashi Yamanami, Takahiro Tsukimura, Hideki Maeda, Tadayasu Togawa, Hitoshi Sakuraba, Satoshi Gojo. Cell Transplantation Combined with Recombinant Collagen Peptides for the Treatment of Fabry Disease.
Cell transplantation.
2020 Jan; 29(?):963689720976362. doi:
10.1177/0963689720976362. [PMID: 33300391] - Magdalena Cerón-Rodríguez, Guillermo Ramón-García, Edgar Barajas-Colón, Isidro Franco-Álvarez, Juan L Salgado-Loza. Renal globotriaosylceramide deposits for Fabry disease linked to uncertain pathogenicity gene variant c.352C>T/p.Arg118Cys: A family study.
Molecular genetics & genomic medicine.
2019 11; 7(11):e981. doi:
10.1002/mgg3.981. [PMID: 31566927] - Wendy E Heywood, Ivan Doykov, Justyna Spiewak, Jenny Hallqvist, Kevin Mills, Albina Nowak. Global glycosphingolipid analysis in urine and plasma of female Fabry disease patients.
Biochimica et biophysica acta. Molecular basis of disease.
2019 10; 1865(10):2726-2735. doi:
10.1016/j.bbadis.2019.07.005. [PMID: 31319156] - S Sestito, F Falvo, A Sallemi, M Petrisano, M G Scuderi, F Tarsitano, G D'Angelo, P Betta, K Roppa, F Parisi, L Pensabene, C Fede, R Chimenz, D Concolino. Renal involvement in paediatric Fabry disease.
Journal of biological regulators and homeostatic agents.
2019 Sep; 33(5 Suppl. 1):59-63. Special Issue: Focus on Pediatric Nephrology. doi:
NULL. [PMID: 31630715] - Ivan Morace, Robert Pilz, Giuseppina Federico, Richard Jennemann, Damir Krunic, Viola Nordström, Johanna von Gerichten, Christian Marsching, Ina Maria Schießl, Johannes Müthing, Christian Wunder, Ludger Johannes, Roger Sandhoff, Hermann-Josef Gröne. Renal globotriaosylceramide facilitates tubular albumin absorption and its inhibition protects against acute kidney injury.
Kidney international.
2019 08; 96(2):327-341. doi:
10.1016/j.kint.2019.02.010. [PMID: 31101366] - Malte Lenders, Franciska Stappers, Christoph Niemietz, Boris Schmitz, Michel Boutin, Paula Johanna Ballmaier, Andree Zibert, Hartmut Schmidt, Stefan-Martin Brand, Christiane Auray-Blais, Eva Brand. Mutation-specific Fabry disease patient-derived cell model to evaluate the amenability to chaperone therapy.
Journal of medical genetics.
2019 08; 56(8):548-556. doi:
10.1136/jmedgenet-2019-106005. [PMID: 31010832] - Anatália Labilloy, Ora A Weisz. Lose the lipid: renoprotection conferred by Gb3 synthase knockout.
Kidney international.
2019 08; 96(2):270-272. doi:
10.1016/j.kint.2019.03.034. [PMID: 31331463] - Renzo Mignani. [The Fabry nephropathy: new insight in diagnosis, monitoring and treatment].
Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologia.
2019 Jul; 36(4):. doi:
NULL. [PMID: 31373466] - Rafaela M Alvariz, Isabel T D S Moreira, Gabriela K Cury, Carmen R Vargas, Alethéa G Barschak. In vitro effect of globotriaosylceramide on electron transport chain complexes and redox parameters.
Anais da Academia Brasileira de Ciencias.
2019 Jun; 91(2):e20181373. doi:
10.1590/0001-3765201920181373. [PMID: 31241709] - Yuya Suzuki, Takao Fukushima, Takahiro Iwasawa, Gen Nakamura, Shigeki Nanasawa, Kunihiko Makino. [Case report of acute encephalopathy caused by enterohemorrhagic Escherichia coli infection in a 24-year-old woman].
Rinsho shinkeigaku = Clinical neurology.
2019 May; 59(5):274-278. doi:
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Molecular genetics and metabolism.
2019 05; 127(1):86-94. doi:
10.1016/j.ymgme.2019.03.010. [PMID: 30987917] - Raphael Schiffmann, Ozlem Goker-Alpan, Myrl Holida, Pilar Giraldo, Laura Barisoni, Robert B Colvin, Charles J Jennette, Gustavo Maegawa, Simeon A Boyadjiev, Derlis Gonzalez, Kathy Nicholls, Ahmad Tuffaha, Mohamed G Atta, Bonita Rup, Martha R Charney, Alona Paz, Mali Szlaifer, Sari Alon, Einat Brill-Almon, Raul Chertkoff, Derralynn Hughes. Pegunigalsidase alfa, a novel PEGylated enzyme replacement therapy for Fabry disease, provides sustained plasma concentrations and favorable pharmacodynamics: A 1-year Phase 1/2 clinical trial.
Journal of inherited metabolic disease.
2019 05; 42(3):534-544. doi:
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American journal of human genetics.
2019 04; 104(4):625-637. doi:
10.1016/j.ajhg.2019.02.003. [PMID: 30879639] - Emma H McCafferty, Lesley J Scott. Migalastat: A Review in Fabry Disease.
Drugs.
2019 Apr; 79(5):543-554. doi:
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Molecular genetics and metabolism.
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Glycobiology.
2019 03; 29(3):260-268. doi:
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Molecular genetics and metabolism.
2019 03; 126(3):212-223. doi:
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Nephron.
2019; 143(4):274-281. doi:
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Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology.
2019; 52(5):1139-1150. doi:
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International journal of medical microbiology : IJMM.
2018 Dec; 308(8):1073-1084. doi:
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Human molecular genetics.
2018 10; 27(19):3392-3403. doi:
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International journal of medical microbiology : IJMM.
2018 Oct; 308(7):940-946. doi:
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Journal of the American Heart Association.
2018 09; 7(17):e009052. doi:
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Molecular genetics and metabolism.
2018 09; 125(1-2):153-160. doi:
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FASEB journal : official publication of the Federation of American Societies for Experimental Biology.
2018 08; 32(8):4544-4559. doi:
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Analytica chimica acta.
2018 Jul; 1015(?):35-49. doi:
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Scientific reports.
2018 07; 8(1):11042. doi:
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Genetics in medicine : official journal of the American College of Medical Genetics.
2018 07; 20(7):754-759. doi:
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Pediatric nephrology (Berlin, Germany).
2018 07; 33(7):1161-1163. doi:
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Medecine et maladies infectieuses.
2018 May; 48(3):167-174. doi:
10.1016/j.medmal.2017.09.012. [PMID: 29054297] - Raphael Schiffmann, Daniel G Bichet, Ana Jovanovic, Derralynn A Hughes, Roberto Giugliani, Ulla Feldt-Rasmussen, Suma P Shankar, Laura Barisoni, Robert B Colvin, J Charles Jennette, Fred Holdbrook, Andrew Mulberg, Jeffrey P Castelli, Nina Skuban, Jay A Barth, Kathleen Nicholls. Migalastat improves diarrhea in patients with Fabry disease: clinical-biomarker correlations from the phase 3 FACETS trial.
Orphanet journal of rare diseases.
2018 04; 13(1):68. doi:
10.1186/s13023-018-0813-7. [PMID: 29703262] - Alana Pimentel Moura, Tatiane Hammerschmidt, Marion Deon, Roberto Giugliani, Carmen Regla Vargas. Investigation of correlation of urinary globotriaosylceramide (Gb3) levels with markers of renal function in patients with Fabry disease.
Clinica chimica acta; international journal of clinical chemistry.
2018 Mar; 478(?):62-67. doi:
10.1016/j.cca.2017.12.033. [PMID: 29274327] - Irene Simonetta, Antonino Tuttolomondo, Tiziana Di Chiara, Salvatore Miceli, Danai Vogiatzis, Francesca Corpora, Antonio Pinto. Genetics and Gene Therapy of Anderson-Fabry Disease.
Current gene therapy.
2018; 18(2):96-106. doi:
10.2174/1566523218666180404161315. [PMID: 29618309] - María Del Pino, Amado Andrés, Ana Ávila Bernabéu, Joaquín de Juan-Rivera, Elvira Fernández, Juan de Dios García Díaz, Domingo Hernández, José Luño, Isabel Martínez Fernández, José Paniagua, Manuel Posada de la Paz, José Carlos Rodríguez-Pérez, Rafael Santamaría, Roser Torra, Joan Torras Ambros, Pedro Vidau, Josep-Vicent Torregrosa. Fabry Nephropathy: An Evidence-Based Narrative Review.
Kidney & blood pressure research.
2018; 43(2):406-421. doi:
10.1159/000488121. [PMID: 29558749] - Johannes Kramer, Frank Weidemann. Biomarkers for Diagnosing and Staging of Fabry Disease.
Current medicinal chemistry.
2018; 25(13):1530-1537. doi:
10.2174/0929867324666170616102112. [PMID: 28618999] - Michel Boutin, Iskren Menkovic, Tristan Martineau, Vanessa Vaillancourt-Lavigueur, Amanda Toupin, Christiane Auray-Blais. Separation and Analysis of Lactosylceramide, Galabiosylceramide, and Globotriaosylceramide by LC-MS/MS in Urine of Fabry Disease Patients.
Analytical chemistry.
2017 12; 89(24):13382-13390. doi:
10.1021/acs.analchem.7b03609. [PMID: 29099167] - Sun Hee Heo, Eungu Kang, Yoon-Myung Kim, Heounjeong Go, Kyung Yong Kim, Jae Yong Jung, Minji Kang, Gu-Hwan Kim, Jae-Min Kim, In-Hee Choi, Jin-Ho Choi, Sung-Chul Jung, Robert J Desnick, Han-Wook Yoo, Beom Hee Lee. Fabry disease: characterisation of the plasma proteome pre- and post-enzyme replacement therapy.
Journal of medical genetics.
2017 11; 54(11):771-780. doi:
10.1136/jmedgenet-2017-104704. [PMID: 28835480] - Paula Rozenfeld, Sandro Feriozzi. Contribution of inflammatory pathways to Fabry disease pathogenesis.
Molecular genetics and metabolism.
2017 11; 122(3):19-27. doi:
10.1016/j.ymgme.2017.09.004. [PMID: 28947349] - Ivan U Kouzel, Gottfried Pohlentz, Julia S Schmitz, Daniel Steil, Hans-Ulrich Humpf, Helge Karch, Johannes Müthing. Shiga Toxin Glycosphingolipid Receptors in Human Caco-2 and HCT-8 Colon Epithelial Cell Lines.
Toxins.
2017 10; 9(11):. doi:
10.3390/toxins9110338. [PMID: 29068380] - Nadine Legros, Gottfried Pohlentz, Jana Runde, Stefanie Dusny, Hans-Ulrich Humpf, Helge Karch, Johannes Müthing. Colocalization of receptors for Shiga toxins with lipid rafts in primary human renal glomerular endothelial cells and influence of D-PDMP on synthesis and distribution of glycosphingolipid receptors.
Glycobiology.
2017 10; 27(10):947-965. doi:
10.1093/glycob/cwx048. [PMID: 28535204] - Rannveig Skrunes, Camilla Tøndel, Sabine Leh, Kristin Kampevold Larsen, Gunnar Houge, Einar Skulstad Davidsen, Carla Hollak, André B P van Kuilenburg, Frédéric M Vaz, Einar Svarstad. Long-Term Dose-Dependent Agalsidase Effects on Kidney Histology in Fabry Disease.
Clinical journal of the American Society of Nephrology : CJASN.
2017 Sep; 12(9):1470-1479. doi:
10.2215/cjn.01820217. [PMID: 28625968] - Kazuya Tsuboi, Hiroshi Yamamoto. Efficacy and safety of enzyme-replacement-therapy with agalsidase alfa in 36 treatment-naïve Fabry disease patients.
BMC pharmacology & toxicology.
2017 06; 18(1):43. doi:
10.1186/s40360-017-0152-7. [PMID: 28592315] - Rannveig Skrunes, Einar Svarstad, Kristin Kampevold Larsen, Sabine Leh, Camilla Tøndel. Reaccumulation of globotriaosylceramide in podocytes after agalsidase dose reduction in young Fabry patients.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.
2017 May; 32(5):807-813. doi:
10.1093/ndt/gfw094. [PMID: 27190352] - Christiane Auray-Blais, Pamela Lavoie, Michel Boutin, Mona Abaoui. High-Risk Screening for Fabry Disease: Analysis by Tandem Mass Spectrometry of Globotriaosylceramide (Gb3 ) in Urine Collected on Filter Paper.
Current protocols in human genetics.
2017 04; 93(?):17.26.1-17.26.12. doi:
10.1002/cphg.34. [PMID: 28384397] - Stefan K Müller, Isabel Wilhelm, Thomas Schubert, Katharina Zittlau, Anne Imberty, Josef Madl, Thorsten Eierhoff, Roland Thuenauer, Winfried Römer. Gb3-binding lectins as potential carriers for transcellular drug delivery.
Expert opinion on drug delivery.
2017 Feb; 14(2):141-153. doi:
10.1080/17425247.2017.1266327. [PMID: 27935765] - Weria Pezeshkian, Haifei Gao, Senthil Arumugam, Ulrike Becken, Patricia Bassereau, Jean-Claude Florent, John Hjort Ipsen, Ludger Johannes, Julian C Shillcock. Mechanism of Shiga Toxin Clustering on Membranes.
ACS nano.
2017 01; 11(1):314-324. doi:
10.1021/acsnano.6b05706. [PMID: 27943675] - Ting-Rong Hsu, Fu-Pang Chang, Tzu-Hung Chu, Shih-Hsien Sung, Svetlana Bizjajeva, Wen-Chung Yu, Dau-Ming Niu. Correlations between Endomyocardial Biopsies and Cardiac Manifestations in Taiwanese Patients with the Chinese Hotspot IVS4+919G>A Mutation: Data from the Fabry Outcome Survey.
International journal of molecular sciences.
2017 Jan; 18(1):. doi:
10.3390/ijms18010119. [PMID: 28075357] - Takashi Kodama, Takahiro Tsukimura, Ikuo Kawashima, Atsuko Sato, Hitoshi Sakuraba, Tadayasu Togawa. Differences in cleavage of globotriaosylceramide and its derivatives accumulated in organs of young Fabry mice following enzyme replacement therapy.
Molecular genetics and metabolism.
2017 Jan; 120(1-2):116-120. doi:
10.1016/j.ymgme.2016.10.003. [PMID: 27756537] - Hong Jai Lee, Hee Ho Park, Youngsoo Sohn, Jina Ryu, Ju Hyun Park, Won Jong Rhee, Tai Hyun Park. α-Galactosidase delivery using 30Kc19-human serum albumin nanoparticles for effective treatment of Fabry disease.
Applied microbiology and biotechnology.
2016 Dec; 100(24):10395-10402. doi:
10.1007/s00253-016-7689-z. [PMID: 27353764] - Mona Abaoui, Michel Boutin, Pamela Lavoie, Christiane Auray-Blais. High-Risk Screening of Fabry Disease: Analysis of Fifteen Urinary Methylated and Non-Methylated Gb3 Isoforms Using Tandem Mass Spectrometry.
Current protocols in human genetics.
2016 10; 91(?):17.24.1-17.24.11. doi:
10.1002/cphg.24. [PMID: 27727434] - Christine R Kaneski, Roscoe O Brady, John A Hanover, Ulrike H Schueler. Development of a model system for neuronal dysfunction in Fabry disease.
Molecular genetics and metabolism.
2016 09; 119(1-2):144-50. doi:
10.1016/j.ymgme.2016.07.010. [PMID: 27471012] - Dominique P Germain, Derralynn A Hughes, Kathleen Nicholls, Daniel G Bichet, Roberto Giugliani, William R Wilcox, Claudio Feliciani, Suma P Shankar, Fatih Ezgu, Hernan Amartino, Drago Bratkovic, Ulla Feldt-Rasmussen, Khan Nedd, Usama Sharaf El Din, Charles M Lourenco, Maryam Banikazemi, Joel Charrow, Majed Dasouki, David Finegold, Pilar Giraldo, Ozlem Goker-Alpan, Nicola Longo, C Ronald Scott, Roser Torra, Ahmad Tuffaha, Ana Jovanovic, Stephen Waldek, Seymour Packman, Elizabeth Ludington, Christopher Viereck, John Kirk, Julie Yu, Elfrida R Benjamin, Franklin Johnson, David J Lockhart, Nina Skuban, Jeff Castelli, Jay Barth, Carrolee Barlow, Raphael Schiffmann. Treatment of Fabry's Disease with the Pharmacologic Chaperone Migalastat.
The New England journal of medicine.
2016 Aug; 375(6):545-55. doi:
10.1056/nejmoa1510198. [PMID: 27509102] - Regina El Dib, Huda Gomaa, Raíssa Pierri Carvalho, Samira E Camargo, Rodrigo Bazan, Pasqual Barretti, Fellype C Barreto. Enzyme replacement therapy for Anderson-Fabry disease.
The Cochrane database of systematic reviews.
2016 Jul; 7(?):CD006663. doi:
10.1002/14651858.cd006663.pub4. [PMID: 27454104] - W Pezeshkian, A G Hansen, L Johannes, H Khandelia, J C Shillcock, P B S Kumar, J H Ipsen. Membrane invagination induced by Shiga toxin B-subunit: from molecular structure to tube formation.
Soft matter.
2016 Jun; 12(23):5164-71. doi:
10.1039/c6sm00464d. [PMID: 27070906] - J Fujii, T Mizoue, T Kita, H Kishimoto, K Joh, Y Nakada, S Ugajin, Y Naya, T Nakamura, Y Tada, N Okabe, Y Maruyama, K Saitoh, Y Kurozawa. Risk of haemolytic uraemic syndrome caused by shiga-toxin-producing Escherichia coli infection in adult women in Japan.
Epidemiology and infection.
2016 Apr; 144(5):952-61. doi:
10.1017/s0950268815002289. [PMID: 26470913] - Fahad J Alharbi, Tarekegn Geberhiwot, Derralynn A Hughes, Douglas G Ward. A Novel Rapid MALDI-TOF-MS-Based Method for Measuring Urinary Globotriaosylceramide in Fabry Patients.
Journal of the American Society for Mass Spectrometry.
2016 Apr; 27(4):719-25. doi:
10.1007/s13361-015-1318-4. [PMID: 26797827] - Takahiro Kanai, Takane Ito, Jun Odaka, Takashi Saito, Jun Aoyagi, Hiroyuki Betsui, Takanori Yamagata. Surges in proteinuria are associated with plasma GL-3 elevations in a young patient with classic Fabry disease.
European journal of pediatrics.
2016 Mar; 175(3):427-31. doi:
10.1007/s00431-015-2646-x. [PMID: 26454753] - Mona Abaoui, Michel Boutin, Pamela Lavoie, Christiane Auray-Blais. Tandem mass spectrometry multiplex analysis of methylated and non-methylated urinary Gb3 isoforms in Fabry disease patients.
Clinica chimica acta; international journal of clinical chemistry.
2016 Jan; 452(?):191-8. doi:
10.1016/j.cca.2015.11.018. [PMID: 26593248] - Martina Gaggl, Natalija Lajic, Georg Heinze, Till Voigtländer, Raute Sunder-Plassmann, Eduard Paschke, Günter Fauler, Gere Sunder-Plassmann, Gerald Mundigler. Screening for Fabry Disease by Urinary Globotriaosylceramide Isoforms Measurement in Patients with Left Ventricular Hypertrophy.
International journal of medical sciences.
2016; 13(5):340-6. doi:
10.7150/ijms.14997. [PMID: 27226774] - K V Firsov, A S Kotov. [The neurological manifestations of Fabry disease. A review].
Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova.
2016 ; 116(9):98-105. doi:
10.17116/jnevro20161169198-105. [PMID: 27735906] - Martina Gaggl, Marlene Hofer, Stefanie Weidner, Julia Kleinert, Günter Fauler, Manfred Wallner, Peter Kotanko, Eduard Paschke, Gere Sunder-Plassmann. Interfering parameters in the determination of urinary globotriaosylceramide (Gb3) in patients with chronic kidney disease.
Journal of nephrology.
2015 Dec; 28(6):679-89. doi:
10.1007/s40620-015-0193-1. [PMID: 25857295] - Daniel Steil, Catherine-Louise Schepers, Gottfried Pohlentz, Nadine Legros, Jana Runde, Hans-Ulrich Humpf, Helge Karch, Johannes Müthing. Shiga toxin glycosphingolipid receptors of Vero-B4 kidney epithelial cells and their membrane microdomain lipid environment.
Journal of lipid research.
2015 Dec; 56(12):2322-36. doi:
10.1194/jlr.m063040. [PMID: 26464281] - Raphael Schiffmann, Caren Swift, Xuan Wang, Derek Blankenship, Markus Ries. A prospective 10-year study of individualized, intensified enzyme replacement therapy in advanced Fabry disease.
Journal of inherited metabolic disease.
2015 Nov; 38(6):1129-36. doi:
10.1007/s10545-015-9845-5. [PMID: 25900714] - Youn-Jeong Shin, Yeo Jin Jeon, Namhee Jung, Joo-Won Park, Hae-Young Park, Sung-Chul Jung. Substrate-specific gene expression profiles in different kidney cell types are associated with Fabry disease.
Molecular medicine reports.
2015 Oct; 12(4):5049-57. doi:
10.3892/mmr.2015.4010. [PMID: 26135632] - Susana Ferreira, Christiane Auray-Blais, Michel Boutin, Pamela Lavoie, José Pedro Nunes, Elisabete Martins, Scott Garman, João Paulo Oliveira. Variations in the GLA gene correlate with globotriaosylceramide and globotriaosylsphingosine analog levels in urine and plasma.
Clinica chimica acta; international journal of clinical chemistry.
2015 Jul; 447(?):96-104. doi:
10.1016/j.cca.2015.06.003. [PMID: 26070511] - F Sacerdoti, M M Amaral, J Aisemberg, C B Cymeryng, A M Franchi, C Ibarra. Involvement of hypoxia and inflammation in early pregnancy loss mediated by Shiga toxin type 2.
Placenta.
2015 Jun; 36(6):674-80. doi:
10.1016/j.placenta.2015.03.005. [PMID: 25819809] - Karen M Ashe, Eva Budman, Dinesh S Bangari, Craig S Siegel, Jennifer B Nietupski, Bing Wang, Robert J Desnick, Ronald K Scheule, John P Leonard, Seng H Cheng, John Marshall. Efficacy of Enzyme and Substrate Reduction Therapy with a Novel Antagonist of Glucosylceramide Synthase for Fabry Disease.
Molecular medicine (Cambridge, Mass.).
2015 Apr; 21(?):389-99. doi:
10.2119/molmed.2015.00088. [PMID: 25938659] - Bouwien E Smid, Linda van der Tol, Marieke Biegstraaten, Gabor E Linthorst, Carla E M Hollak, Ben J H M Poorthuis. Plasma globotriaosylsphingosine in relation to phenotypes of Fabry disease.
Journal of medical genetics.
2015 Apr; 52(4):262-8. doi:
10.1136/jmedgenet-2014-102872. [PMID: 25596309] - Ida Arvidsson, Anne-Lie Ståhl, Minola Manea Hedström, Ann-Charlotte Kristoffersson, Christian Rylander, Julia S Westman, Jill R Storry, Martin L Olsson, Diana Karpman. Shiga toxin-induced complement-mediated hemolysis and release of complement-coated red blood cell-derived microvesicles in hemolytic uremic syndrome.
Journal of immunology (Baltimore, Md. : 1950).
2015 Mar; 194(5):2309-18. doi:
10.4049/jimmunol.1402470. [PMID: 25637016] - Weria Pezeshkian, Vitaly V Chaban, Ludger Johannes, Julian Shillcock, John H Ipsen, Himanshu Khandelia. The effects of globotriaosylceramide tail saturation level on bilayer phases.
Soft matter.
2015 Feb; 11(7):1352-61. doi:
10.1039/c4sm02456g. [PMID: 25575293] - Hideaki Sueoka, Mikio Aoki, Takahiro Tsukimura, Tadayasu Togawa, Hitoshi Sakuraba. Distributions of Globotriaosylceramide Isoforms, and Globotriaosylsphingosine and Its Analogues in an α-Galactosidase A Knockout Mouse, a Model of Fabry Disease.
PloS one.
2015; 10(12):e0144958. doi:
10.1371/journal.pone.0144958. [PMID: 26661087] - Yeo Jin Jeon, Namhee Jung, Joo-Won Park, Hae-Young Park, Sung-Chul Jung. Epithelial-Mesenchymal Transition in Kidney Tubular Epithelial Cells Induced by Globotriaosylsphingosine and Globotriaosylceramide.
PloS one.
2015; 10(8):e0136442. doi:
10.1371/journal.pone.0136442. [PMID: 26291612]