(E)-N-[(E)-1-[5-[3,4-dihydroxy-6-(hydroxymethyl)-5-[3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxyoxan-2-yl]oxy-3,4-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy-3-hydroxyoctadec-4-en-2-yl]hexacos-17-enamide (BioDeep_00000913220)
代谢物信息卡片
(E)-N-[(E)-1-[5-[3,4-dihydroxy-6-(hydroxymethyl)-5-[3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxyoxan-2-yl]oxy-3,4-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy-3-hydroxyoctadec-4-en-2-yl]hexacos-17-enamide
化学式: C62H115NO18 (1161.811373)
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)CCCCCCCCCCCCCCCC=CCCCCCCCC)O
InChI: InChI=1S/C62H115NO18/c1-3-5-7-9-11-13-15-17-18-19-20-21-22-23-24-25-26-28-30-32-34-36-38-40-50(68)63-45(46(67)39-37-35-33-31-29-27-16-14-12-10-8-6-4-2)44-76-60-56(74)53(71)58(48(42-65)78-60)81-62-57(75)54(72)59(49(43-66)79-62)80-61-55(73)52(70)51(69)47(41-64)77-61/h17-18,37,39,45-49,51-62,64-67,69-75H,3-16,19-36,38,40-44H2,1-2H3,(H,63,68)/b18-17+,39-37+
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
- Pankaj Sharma, Xiaolong Zhang, Kevin Ly, Yuxiang Zhang, Yu Hu, Adam Yongxin Ye, Jianqiao Hu, Ji Hyung Kim, Mumeng Lou, Chong Wang, Quinton Celuzza, Yuji Kondo, Keiko Furukawa, David R Bundle, Koichi Furukawa, Frederick W Alt, Florian Winau. The lipid globotriaosylceramide promotes germinal center B cell responses and antiviral immunity.
Science (New York, N.Y.).
2024 Feb; 383(6684):eadg0564. doi:
10.1126/science.adg0564. [PMID: 38359115] - 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:
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Clinical and experimental nephrology.
2021 Sep; 25(9):925-934. doi:
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Analytical biochemistry.
2021 09; 628(?):114287. doi:
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Toxins.
2021 07; 13(8):. doi:
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Journal of inherited metabolic disease.
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Biochemical and biophysical research communications.
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Toxins.
2021 05; 13(6):. doi:
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Nature communications.
2021 05; 12(1):2673. doi:
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Cellular and molecular life sciences : CMLS.
2021 Apr; 78(7):3637-3656. doi:
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Molecular genetics and metabolism.
2021 04; 132(4):234-243. doi:
10.1016/j.ymgme.2021.02.001. [PMID: 33642210] - 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] - Junya Sugimoto, Hiroshi Satoyoshi, Kazue Takahata, Shizuko Muraoka. Fabry disease-associated globotriaosylceramide induces mechanical allodynia via activation of signaling through proNGF-p75NTR but not mature NGF-TrkA.
European journal of pharmacology.
2021 Mar; 895(?):173882. doi:
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Nature communications.
2021 02; 12(1):1178. doi:
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Toxins.
2021 02; 13(2):. doi:
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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:
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Brain : a journal of neurology.
2020 12; 143(11):3331-3342. doi:
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Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir.
2020 12; 48(Suppl 2):1-42. doi:
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Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie.
2020 Nov; 131(?):110779. doi:
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Journal of inherited metabolic disease.
2020 09; 43(5):922-933. doi:
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International journal of molecular sciences.
2020 Aug; 21(17):. doi:
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Scientific reports.
2020 06; 10(1):9752. doi:
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The Journal of biological chemistry.
2020 04; 295(17):5577-5587. doi:
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Journal of the American Society of Nephrology : JASN.
2020 04; 31(4):865-875. doi:
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The Journal of biological chemistry.
2020 03; 295(13):4341-4349. doi:
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CEN case reports.
2020 02; 9(1):24-29. doi:
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Clinica chimica acta; international journal of clinical chemistry.
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Revista da Associacao Medica Brasileira (1992).
2020 Jan; 66Suppl 1(Suppl 1):s10-s16. doi:
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Cell transplantation.
2020 Jan; 29(?):963689720976362. doi:
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Molecular genetics & genomic medicine.
2019 11; 7(11):e981. doi:
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Nano letters.
2019 10; 19(10):7365-7369. doi:
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Biochimica et biophysica acta. Molecular basis of disease.
2019 10; 1865(10):2726-2735. doi:
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Journal of biological regulators and homeostatic agents.
2019 Sep; 33(5 Suppl. 1):59-63. Special Issue: Focus on Pediatric Nephrology. doi:
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Kidney international.
2019 08; 96(2):270-272. doi:
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Journal of medical genetics.
2019 08; 56(8):548-556. doi:
10.1136/jmedgenet-2019-106005. [PMID: 31010832] - 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:
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Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologia.
2019 Jul; 36(4):. doi:
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Anais da Academia Brasileira de Ciencias.
2019 Jun; 91(2):e20181373. doi:
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Rinsho shinkeigaku = Clinical neurology.
2019 May; 59(5):274-278. doi:
10.5692/clinicalneurol.cn-001291. [PMID: 31061306] - 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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Molecular genetics and metabolism.
2019 05; 127(1):86-94. doi:
10.1016/j.ymgme.2019.03.010. [PMID: 30987917] - Xuling Zhu, Ling Yin, Matt Theisen, Jenny Zhuo, Summar Siddiqui, Becca Levy, Vladimir Presnyak, Andrea Frassetto, Jaclyn Milton, Timothy Salerno, Kerry E Benenato, Joe Milano, Andy Lynn, Staci Sabnis, Kristine Burke, Gilles Besin, Christine M Lukacs, Lin T Guey, Patrick F Finn, Paolo G V Martini. Systemic mRNA Therapy for the Treatment of Fabry Disease: Preclinical Studies in Wild-Type Mice, Fabry Mouse Model, and Wild-Type Non-human Primates.
American journal of human genetics.
2019 04; 104(4):625-637. doi:
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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:
10.1093/glycob/cwy105. [PMID: 30476082] - Dominique P Germain, Michael Arad, Alessandro Burlina, Perry M Elliott, Bruno Falissard, Ulla Feldt-Rasmussen, Max J Hilz, Derralynn A Hughes, Alberto Ortiz, Christoph Wanner, Frank Weidemann, Marco Spada. The effect of enzyme replacement therapy on clinical outcomes in female patients with Fabry disease - A systematic literature review by a European panel of experts.
Molecular genetics and metabolism.
2019 03; 126(3):224-235. 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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Cell research.
2019 01; 29(1):42-53. doi:
10.1038/s41422-018-0122-7. [PMID: 30514903] - Judith Reindl, Maxim Shevtsov, Günther Dollinger, Stefan Stangl, Gabriele Multhoff. Membrane Hsp70-supported cell-to-cell connections via tunneling nanotubes revealed by live-cell STED nanoscopy.
Cell stress & chaperones.
2019 01; 24(1):213-221. doi:
10.1007/s12192-018-00958-w. [PMID: 30632067] - Nadine Legros, Gottfried Pohlentz, Daniel Steil, Johannes Müthing. Shiga toxin-glycosphingolipid interaction: Status quo of research with focus on primary human brain and kidney endothelial cells.
International journal of medical microbiology : IJMM.
2018 Dec; 308(8):1073-1084. doi:
10.1016/j.ijmm.2018.09.003. [PMID: 30224239] - Songhai Tian, Khaja Muneeruddin, Mei Yuk Choi, Liang Tao, Robiul H Bhuiyan, Yuhsuke Ohmi, Keiko Furukawa, Koichi Furukawa, Sebastian Boland, Scott A Shaffer, Rosalyn M Adam, Min Dong. Genome-wide CRISPR screens for Shiga toxins and ricin reveal Golgi proteins critical for glycosylation.
PLoS biology.
2018 11; 16(11):e2006951. doi:
10.1371/journal.pbio.2006951. [PMID: 30481169] - Maurizio Brigotti, Domenica Carnicelli, Valentina Arfilli, Elisa Porcellini, Elisabetta Galassi, Maria C Valerii, Enzo Spisni. Human monocytes stimulated by Shiga toxin 1a via globotriaosylceramide release proinflammatory molecules associated with hemolytic uremic syndrome.
International journal of medical microbiology : IJMM.
2018 Oct; 308(7):940-946. doi:
10.1016/j.ijmm.2018.06.013. [PMID: 29983334] - R W D Welford, A Mühlemann, M Garzotti, V Rickert, P M A Groenen, O Morand, N Üçeyler, M R Probst. Glucosylceramide synthase inhibition with lucerastat lowers globotriaosylceramide and lysosome staining in cultured fibroblasts from Fabry patients with different mutation types.
Human molecular genetics.
2018 10; 27(19):3392-3403. doi:
10.1093/hmg/ddy248. [PMID: 29982630] - Andrea Frustaci, Romina Verardo, Claudia Grande, Nicola Galea, Pierluca Piselli, Iacopo Carbone, Maria Alfarano, Matteo Antonio Russo, Cristina Chimenti. Immune-Mediated Myocarditis in Fabry Disease Cardiomyopathy.
Journal of the American Heart Association.
2018 09; 7(17):e009052. doi:
10.1161/jaha.118.009052. [PMID: 30371172] - Hideto Morimoto, Yae Ito, Eiji Yoden, Masato Horie, Noboru Tanaka, Yoshikazu Komurasaki, Ryuji Yamamoto, Kazutoshi Mihara, Kohtaro Minami, Tohru Hirato. Non-clinical evaluation of JR-051 as a biosimilar to agalsidase beta for the treatment of Fabry disease.
Molecular genetics and metabolism.
2018 09; 125(1-2):153-160. doi:
10.1016/j.ymgme.2018.07.009. [PMID: 30054149] - Hiroki Maruyama, Atsumi Taguchi, Yuji Nishikawa, Chu Guili, Mariko Mikame, Masaaki Nameta, Yutaka Yamaguchi, Mitsuhiro Ueno, Naofumi Imai, Yumi Ito, Takahiko Nakagawa, Ichiei Narita, Satoshi Ishii. Medullary thick ascending limb impairment in the GlatmTg(CAG-A4GALT) Fabry model mice.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology.
2018 08; 32(8):4544-4559. doi:
10.1096/fj.201701374r. [PMID: 29553830] - Amanda Toupin, Pamela Lavoie, Marie-Françoise Arthus, Mona Abaoui, Michel Boutin, Carole Fortier, Claudia Ménard, Daniel G Bichet, Christiane Auray-Blais. Analysis of globotriaosylceramide (Gb3) isoforms/analogs in unfractionated leukocytes, B lymphocytes and monocytes from Fabry patients using ultra-high performance liquid chromatography/tandem mass spectrometry.
Analytica chimica acta.
2018 Jul; 1015(?):35-49. doi:
10.1016/j.aca.2018.02.022. [PMID: 29530250] - Gisela G Slaats, Fabian Braun, Martin Hoehne, Laura E Frech, Linda Blomberg, Thomas Benzing, Bernhard Schermer, Markus M Rinschen, Christine E Kurschat. Urine-derived cells: a promising diagnostic tool in Fabry disease patients.
Scientific reports.
2018 07; 8(1):11042. doi:
10.1038/s41598-018-29240-w. [PMID: 30038331] - Gülşah Kaya Aksoy, Elif Çomak, Bahar Akkaya, Mustafa Koyun, Sema Akman. Proteinuria in a male adolescent with hearing loss: Answers.
Pediatric nephrology (Berlin, Germany).
2018 07; 33(7):1161-1163. doi:
10.1007/s00467-017-3825-y. [PMID: 29098401] - Raphael Schiffmann, Caren Swift, Nathan McNeill, Elfrida R Benjamin, Jeffrey P Castelli, Jay Barth, Lawrence Sweetman, Xuan Wang, Xiaoyang Wu. Low frequency of Fabry disease in patients with common heart disease.
Genetics in medicine : official journal of the American College of Medical Genetics.
2018 07; 20(7):754-759. doi:
10.1038/gim.2017.175. [PMID: 29227985] - M Bruyand, P Mariani-Kurkdjian, M Gouali, H de Valk, L A King, S Le Hello, S Bonacorsi, C Loirat. Hemolytic uremic syndrome due to Shiga toxin-producing Escherichia coli infection.
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] - 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] - 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] - Einar Svarstad, Sabine Leh, Rannveig Skrunes, Kristin Kampevold Larsen, Øystein Eikrem, Camilla Tøndel. Bedside Stereomicroscopy of Fabry Kidney Biopsies: An Easily Available Method for Diagnosis and Assessment of Sphingolipid Deposits.
Nephron.
2018; 138(1):13-21. doi:
10.1159/000479751. [PMID: 28848191] - 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] - 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] - 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] - 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] - Pavel Volynsky, Roman Efremov, Ilya Mikhalev, Kira Dobrochaeva, Alexander Tuzikov, Elena Korchagina, Polina Obukhova, Evgenia Rapoport, Nicolai Bovin. Why human anti-Galα1-4Galβ1-4Glc natural antibodies do not recognize the trisaccharide on erythrocyte membrane? Molecular dynamics and immunochemical investigation.
Molecular immunology.
2017 10; 90(?):87-97. doi:
10.1016/j.molimm.2017.06.247. [PMID: 28708979] - 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] - Shuangshuang Zheng, Thorsten Eierhoff, Sahaja Aigal, Annette Brandel, Roland Thuenauer, Sophie de Bentzmann, Anne Imberty, Winfried Römer. The Pseudomonas aeruginosa lectin LecA triggers host cell signalling by glycosphingolipid-dependent phosphorylation of the adaptor protein CrkII.
Biochimica et biophysica acta. Molecular cell research.
2017 Jul; 1864(7):1236-1245. doi:
10.1016/j.bbamcr.2017.04.005. [PMID: 28428058] - 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] - Graham Brogden, Hadeel Shammas, Katia Maalouf, Samara L Naim, Gabi Wetzel, Mahdi Amiri, Maren von Köckritz-Blickwede, Anibh M Das, Hassan Y Naim. Case study on the pathophysiology of Fabry disease: abnormalities of cellular membranes can be reversed by substrate reduction in vitro.
Bioscience reports.
2017 04; 37(2):. doi:
10.1042/bsr20160402. [PMID: 28351893] - 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] - Nadine Legros, Stefanie Dusny, Hans-Ulrich Humpf, Gottfried Pohlentz, Helge Karch, Johannes Müthing. Shiga toxin glycosphingolipid receptors and their lipid membrane ensemble in primary human blood-brain barrier endothelial cells.
Glycobiology.
2017 01; 27(1):99-109. doi:
10.1093/glycob/cww090. [PMID: 27558838] - 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] - Xing-Li Meng, Taniqua S Day, Nathan McNeill, Paula Ashcraft, Thomas Frischmuth, Seng H Cheng, Zhi-Ping Liu, Jin-Song Shen, Raphael Schiffmann. Molecular basis for globotriaosylceramide regulation and enzyme uptake in immortalized aortic endothelial cells from Fabry mice.
Journal of inherited metabolic disease.
2016 05; 39(3):447-455. doi:
10.1007/s10545-016-9920-6. [PMID: 26960552] - 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] - 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]