Migalastat hydrochloride (BioDeep_00000759955)
代谢物信息卡片
Migalastat hydrochloride
化学式: C6H14ClNO4 (199.0611314)
中文名称: 盐酸脱氧半乳糖嘧啶
谱图信息:
最多检出来源 () 0%
分子结构信息
SMILES: C1C(C(C(C(N1)CO)O)O)O.Cl
InChI: /m1./s1
相关代谢途径
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代谢反应
0 个相关的代谢反应过程信息。
Reactome(0)
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Plant Reactome(0)
INOH(0)
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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。
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文献列表
- Malte Lenders, Peter Nordbeck, Christine Kurschat, Maria Eveslage, Nesrin Karabul, Jessica Kaufeld, Julia B Hennermann, Monica Patten, Markus Cybulla, Jonas Müntze, Nurcan Üçeyler, Dan Liu, Anibh M Das, Claudia Sommer, Christian Pogoda, Stefanie Reiermann, Thomas Duning, Jens Gaedeke, Katharina von Cossel, Daniela Blaschke, Stefan-Martin Brand, W Alexander Mann, Christoph Kampmann, Nicole Muschol, Sima Canaan-Kühl, Eva Brand. Treatment of Fabry Disease management with migalastat-outcome from a prospective 24 months observational multicenter study (FAMOUS).
European heart journal. Cardiovascular pharmacotherapy.
2022 05; 8(3):272-281. doi:
10.1093/ehjcvp/pvab025. [PMID: 35512362] - Masato Minami, Emiko Mizuma, Mai Nakahara, Yumi Oda, Haruhito Yoshimine, Koki Tokunaga, Akihiko Mitsuke, Yasutoshi Yamada, Hideki Enokida, Kosuke Masutani, Norihiko Goto, Akio Ido. A case of latent heterozygous Fabry disease in a female living kidney donor candidate.
CEN case reports.
2021 02; 10(1):30-34. doi:
10.1007/s13730-020-00510-9. [PMID: 32712909] - Daniel G Bichet, Johannes M Aerts, Christiane Auray-Blais, Hiroki Maruyama, Atul B Mehta, Nina Skuban, Eva Krusinska, Raphael Schiffmann. Assessment of plasma lyso-Gb3 for clinical monitoring of treatment response in migalastat-treated patients with Fabry disease.
Genetics in medicine : official journal of the American College of Medical Genetics.
2021 01; 23(1):192-201. doi:
10.1038/s41436-020-00968-z. [PMID: 32994552] - Eleonora Riccio, Mario Zanfardino, Lucia Ferreri, Ciro Santoro, Sirio Cocozza, Ivana Capuano, Massimo Imbriaco, Sandro Feriozzi, Antonio Pisani. Switch from enzyme replacement therapy to oral chaperone migalastat for treating fabry disease: real-life data.
European journal of human genetics : EJHG.
2020 12; 28(12):1662-1668. doi:
10.1038/s41431-020-0677-x. [PMID: 32647377] - Markus S Mueller, Patricia N Sidharta, Christine Voors-Pette, Borje Darpo, Hongqi Xue, Jasper Dingemanse. The effect of the glucosylceramide synthase inhibitor lucerastat on cardiac repolarization: results from a thorough QT study in healthy subjects.
Orphanet journal of rare diseases.
2020 10; 15(1):303. doi:
10.1186/s13023-020-01582-7. [PMID: 33109218] - Hila Braunstein, Maria Papazian, Gali Maor, Jan Lukas, Arndt Rolfs, Mia Horowitz. Misfolding of Lysosomal α-Galactosidase a in a Fly Model and Its Alleviation by the Pharmacological Chaperone Migalastat.
International journal of molecular sciences.
2020 Oct; 21(19):. doi:
10.3390/ijms21197397. [PMID: 33036426] - Ulla Feldt-Rasmussen, Derralynn Hughes, Gere Sunder-Plassmann, Suma Shankar, Khan Nedd, Iacopo Olivotto, Damara Ortiz, Toya Ohashi, Takashi Hamazaki, Nina Skuban, Julie Yu, Jay A Barth, Kathleen Nicholls. Long-term efficacy and safety of migalastat treatment in Fabry disease: 30-month results from the open-label extension of the randomized, phase 3 ATTRACT study.
Molecular genetics and metabolism.
2020 Sep; 131(1-2):219-228. doi:
10.1016/j.ymgme.2020.07.007. [PMID: 33012654] - Malte Lenders, Peter Nordbeck, Christine Kurschat, Nesrin Karabul, Jessica Kaufeld, Julia B Hennermann, Monica Patten, Markus Cybulla, Jonas Müntze, Nurcan Üçeyler, Dan Liu, Anibh M Das, Claudia Sommer, Christian Pogoda, Stefanie Reiermann, Thomas Duning, Jens Gaedeke, Katharina Stumpfe, Daniela Blaschke, Stefan-Martin Brand, W Alexander Mann, Christoph Kampmann, Nicole Muschol, Sima Canaan-Kühl, Eva Brand. Treatment of Fabry's Disease With Migalastat: Outcome From a Prospective Observational Multicenter Study (FAMOUS).
Clinical pharmacology and therapeutics.
2020 08; 108(2):326-337. doi:
10.1002/cpt.1832. [PMID: 32198894] - Marie-Laure Boof, Atef Halabi, Mike Ufer, Jasper Dingemanse. Impact of the organic cation transporter 2 inhibitor cimetidine on the single-dose pharmacokinetics of the glucosylceramide synthase inhibitor lucerastat in healthy subjects.
European journal of clinical pharmacology.
2020 Mar; 76(3):431-437. doi:
10.1007/s00228-019-02808-9. [PMID: 31836927] - Ichiei Narita, Toya Ohashi, Norio Sakai, Takashi Hamazaki, Nina Skuban, Jeffrey P Castelli, Hjalmar Lagast, Jay A Barth. Efficacy and safety of migalastat in a Japanese population: a subgroup analysis of the ATTRACT study.
Clinical and experimental nephrology.
2020 Feb; 24(2):157-166. doi:
10.1007/s10157-019-01810-w. [PMID: 31889231] - Patrícia Varela, Gianna Mastroianni Kirsztajn, Henrique Ferrer, Carolina Aranda, Krissia Wallbach, Gustavo Ferreira da Mata, Luiz A Moura, Sílvia R Moreira, Carmen Mendes, Marco A Curiati, Ana Maria Martins, João Bosco Pesquero. Functional Characterization and Pharmacological Evaluation of a Novel GLA Missense Mutation Found in a Severely Affected Fabry Disease Family.
Nephron.
2020; 144(3):147-155. doi:
10.1159/000503998. [PMID: 31665721] - Vijayalaxmi Gupta, Sheri A Hild, Sudhakar R Jakkaraj, Erick J Carlson, Henry L Wong, C. Leigh Allen, Gunda I Georg, Joseph S Tash. N-Butyldeoxygalactonojirimycin Induces Reversible Infertility in Male CD Rats.
International journal of molecular sciences.
2019 12; 21(1):. doi:
10.3390/ijms21010301. [PMID: 31906257] - Dominique P Germain, Kathy Nicholls, Roberto Giugliani, Daniel G Bichet, Derralynn A Hughes, Laura M Barisoni, Robert B Colvin, J Charles Jennette, Nina Skuban, Jeffrey P Castelli, Elfrida Benjamin, Jay A Barth, Christopher Viereck. Efficacy of the pharmacologic chaperone migalastat in a subset of male patients with the classic phenotype of Fabry disease and migalastat-amenable variants: data from the phase 3 randomized, multicenter, double-blind clinical trial and extension study.
Genetics in medicine : official journal of the American College of Medical Genetics.
2019 09; 21(9):1987-1997. doi:
10.1038/s41436-019-0451-z. [PMID: 30723321] - Foudil Lamari, Wladimir Mauhin, Fairouz Koraichi, Walid Khrouf, Celine Bordet, Jonathan London, Olivier Lidove, Philippe Charron. Strong increase of leukocyte apha-galactosidase A activity in two male patients with Fabry disease following oral chaperone therapy.
Molecular genetics & genomic medicine.
2019 09; 7(9):e894. doi:
10.1002/mgg3.894. [PMID: 31393666] - 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] - 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] - Susan Oommen, Yanfeng Zhou, Muthuraman Meiyappan, Andrey Gurevich, Yongchang Qiu. Inter-assay variability influences migalastat amenability assessments among Fabry disease variants.
Molecular genetics and metabolism.
2019 05; 127(1):74-85. doi:
10.1016/j.ymgme.2019.04.005. [PMID: 31036492] - Jonas Müntze, Daniel Gensler, Octavian Maniuc, Dan Liu, Tereza Cairns, Daniel Oder, Kai Hu, Kristina Lorenz, Stefan Frantz, Christoph Wanner, Peter Nordbeck. Oral Chaperone Therapy Migalastat for Treating Fabry Disease: Enzymatic Response and Serum Biomarker Changes After 1 Year.
Clinical pharmacology and therapeutics.
2019 05; 105(5):1224-1233. doi:
10.1002/cpt.1321. [PMID: 30506669] - Emma H McCafferty, Lesley J Scott. Migalastat: A Review in Fabry Disease.
Drugs.
2019 Apr; 79(5):543-554. doi:
10.1007/s40265-019-01090-4. [PMID: 30875019] - Chi Zhou, Jin Huang, Guanglin Cui, Hesong Zeng, Dao Wen Wang, Qiang Zhou. Identification of a novel loss-of-function mutation of the GLA gene in a Chinese Han family with Fabry disease.
BMC medical genetics.
2018 12; 19(1):219. doi:
10.1186/s12881-018-0734-2. [PMID: 30587147] - 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] - 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] - Nicolas Guérard, Daniel Oder, Peter Nordbeck, Christian Zwingelstein, Olivier Morand, Richard W D Welford, Jasper Dingemanse, Christoph Wanner. Lucerastat, an Iminosugar for Substrate Reduction Therapy: Tolerability, Pharmacodynamics, and Pharmacokinetics in Patients With Fabry Disease on Enzyme Replacement.
Clinical pharmacology and therapeutics.
2018 04; 103(4):703-711. doi:
10.1002/cpt.790. [PMID: 28699267] - 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] - Nicolas Guérard, Christian Zwingelstein, Jasper Dingemanse. Lucerastat, an Iminosugar for Substrate Reduction Therapy: Pharmacokinetics, Tolerability, and Safety in Subjects With Mild, Moderate, and Severe Renal Function Impairment.
Journal of clinical pharmacology.
2017 Nov; 57(11):1425-1431. doi:
10.1002/jcph.944. [PMID: 28618006] - Elfrida R Benjamin, Maria Cecilia Della Valle, Xiaoyang Wu, Evan Katz, Farhana Pruthi, Sarah Bond, Benjamin Bronfin, Hadis Williams, Julie Yu, Daniel G Bichet, Dominique P Germain, Roberto Giugliani, Derralynn Hughes, Raphael Schiffmann, William R Wilcox, Robert J Desnick, John Kirk, Jay Barth, Carrolee Barlow, Kenneth J Valenzano, Jeff Castelli, David J Lockhart. The validation of pharmacogenetics for the identification of Fabry patients to be treated with migalastat.
Genetics in medicine : official journal of the American College of Medical Genetics.
2017 04; 19(4):430-438. doi:
10.1038/gim.2016.122. [PMID: 27657681] - Derralynn A Hughes, Kathleen Nicholls, Suma P Shankar, Gere Sunder-Plassmann, David Koeller, Khan Nedd, Gerard Vockley, Takashi Hamazaki, Robin Lachmann, Toya Ohashi, Iacopo Olivotto, Norio Sakai, Patrick Deegan, David Dimmock, François Eyskens, Dominique P Germain, Ozlem Goker-Alpan, Eric Hachulla, Ana Jovanovic, Charles M Lourenco, Ichiei Narita, Mark Thomas, William R Wilcox, Daniel G Bichet, Raphael Schiffmann, Elizabeth Ludington, Christopher Viereck, John Kirk, Julie Yu, Franklin Johnson, Pol Boudes, Elfrida R Benjamin, David J Lockhart, Carrolee Barlow, Nina Skuban, Jeffrey P Castelli, Jay Barth, Ulla Feldt-Rasmussen. Oral pharmacological chaperone migalastat compared with enzyme replacement therapy in Fabry disease: 18-month results from the randomised phase III ATTRACT study.
Journal of medical genetics.
2017 04; 54(4):288-296. doi:
10.1136/jmedgenet-2016-104178. [PMID: 27834756] - 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] - Franklin K Johnson, Paul N Mudd, Tara DiMino, Jennie Vosk, Sheela Sitaraman, Pol Boudes, Nicholas France, Carrolee Barlow. An open-label study to determine the pharmacokinetics and safety of migalastat HCl in subjects with impaired renal function and healthy subjects with normal renal function.
Clinical pharmacology in drug development.
2015 07; 4(4):256-61. doi:
10.1002/cpdd.149. [PMID: 27136905] - David G Warnock, Daniel G Bichet, Myrl Holida, Ozlem Goker-Alpan, Kathy Nicholls, Mark Thomas, Francois Eyskens, Suma Shankar, Mathews Adera, Sheela Sitaraman, Richie Khanna, John J Flanagan, Brandon A Wustman, Jay Barth, Carrolee Barlow, Kenneth J Valenzano, David J Lockhart, Pol Boudes, Franklin K Johnson. Oral Migalastat HCl Leads to Greater Systemic Exposure and Tissue Levels of Active α-Galactosidase A in Fabry Patients when Co-Administered with Infused Agalsidase.
PloS one.
2015; 10(8):e0134341. doi:
10.1371/journal.pone.0134341. [PMID: 26252393] - Christina M Ridley, Karen E Thur, Jessica Shanahan, Nagendra Babu Thillaiappan, Ann Shen, Karly Uhl, Charlotte M Walden, Ahad A Rahim, Simon N Waddington, Frances M Platt, Aarnoud C van der Spoel. β-Glucosidase 2 (GBA2) activity and imino sugar pharmacology.
The Journal of biological chemistry.
2013 Sep; 288(36):26052-26066. doi:
10.1074/jbc.m113.463562. [PMID: 23880767] - R Giugliani, S Waldek, D P Germain, K Nicholls, D G Bichet, J K Simosky, A C Bragat, J P Castelli, E R Benjamin, P F Boudes. A Phase 2 study of migalastat hydrochloride in females with Fabry disease: selection of population, safety and pharmacodynamic effects.
Molecular genetics and metabolism.
2013 May; 109(1):86-92. doi:
10.1016/j.ymgme.2013.01.009. [PMID: 23474038] - Jan Lukas, Anne-Katrin Giese, Arseni Markoff, Ulrike Grittner, Ed Kolodny, Hermann Mascher, Karl J Lackner, Wolfgang Meyer, Phillip Wree, Viatcheslav Saviouk, Arndt Rolfs. Functional characterisation of alpha-galactosidase a mutations as a basis for a new classification system in fabry disease.
PLoS genetics.
2013; 9(8):e1003632. doi:
10.1371/journal.pgen.1003632. [PMID: 23935525] - Brandy Young-Gqamana, Nastry Brignol, Hui-Hwa Chang, Richie Khanna, Rebecca Soska, Maria Fuller, Sheela A Sitaraman, Dominique P Germain, Roberto Giugliani, Derralynn A Hughes, Atul Mehta, Kathy Nicholls, Pol Boudes, David J Lockhart, Kenneth J Valenzano, Elfrida R Benjamin. Migalastat HCl reduces globotriaosylsphingosine (lyso-Gb3) in Fabry transgenic mice and in the plasma of Fabry patients.
PloS one.
2013; 8(3):e57631. doi:
10.1371/journal.pone.0057631. [PMID: 23472096] - Chikara Shiozuka, Atsumi Taguchi, Junichiro Matsuda, Yoko Noguchi, Takanori Kunieda, Kozue Uchio-Yamada, Hidekatsu Yoshioka, Ryoji Hamanaka, Shinji Yano, Shigeo Yokoyama, Kazuaki Mannen, Ashok B Kulkarni, Koichi Furukawa, Satoshi Ishii. Increased globotriaosylceramide levels in a transgenic mouse expressing human alpha1,4-galactosyltransferase and a mouse model for treating Fabry disease.
Journal of biochemistry.
2011 Feb; 149(2):161-70. doi:
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Journal of neurochemistry.
2010 Jun; 113(6):1525-35. doi:
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Molecular therapy : the journal of the American Society of Gene Therapy.
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Journal of plant physiology.
2009 Aug; 166(12):1329-1335. doi:
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The Journal of pharmacology and experimental therapeutics.
2009 Mar; 328(3):723-31. doi:
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Molecular genetics and metabolism.
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Molecular biology of the cell.
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Journal of lipid research.
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Annals of neurology.
2004 Nov; 56(5):642-9. doi:
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The Journal of nutrition.
2003 Mar; 133(3):773-6. doi:
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Journal de la Societe de biologie.
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