Eliglustat Tartrate (BioDeep_00000015312)
代谢物信息卡片
Eliglustat hemitartrate
化学式: C50H78N4O14 (958.5514248)
中文名称: 依利格鲁司特酒石酸盐
谱图信息:
最多检出来源 () 0%
分子结构信息
SMILES: CCCCCCCC(=O)NC(CN1CCCC1)C(C2=CC3=C(C=C2)OCCO3)O.CCCCCCCC(=O)NC(CN1CCCC1)C(C2=CC3=C(C=C2)OCCO3)O.C(C(C(=O)O)O)(C(=O)O)O
InChI: 1-,2-/m111/s1
描述信息
A - Alimentary tract and metabolism > A16 - Other alimentary tract and metabolism products > A16A - Other alimentary tract and metabolism products > A16AB - Enzymes
D004791 - Enzyme Inhibitors
C471 - Enzyme Inhibitor
同义名列表
2 个代谢物同义名
数据库引用编号
6 个数据库交叉引用编号
- ChEBI: CHEBI:83353
- KEGG: C16736
- KEGGdrug: D09894
- PubChem: 52918379
- CAS: 928659-70-5
- CAS: 104138-64-9
分类词条
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
- Houfu Leng, Anna Katharina Simon, Nicole J Horwood. Blocking glycosphingolipid production alters autophagy in osteoclasts and improves myeloma bone disease.
Autophagy.
2023 Sep; ?(?):1-3. doi:
10.1080/15548627.2023.2208931. [PMID: 37771244] - M Judith Peterschmitt, Meredith C Foster, Allena J Ji, Marianne B Zajdel, Gerald F Cox. Plasma glucosylsphingosine correlations with baseline disease burden and response to eliglustat in two clinical trials of previously untreated adults with Gaucher disease type 1.
Molecular genetics and metabolism.
2023 Jan; 138(3):107527. doi:
10.1016/j.ymgme.2023.107527. [PMID: 36739645] - Daiana S Sánchez, Lilian K Fischer Sigel, Alejandro Balestracci, Cristina Ibarra, María M Amaral, Claudia Silberstein. Eliglustat prevents Shiga toxin 2 cytotoxic effects in human renal tubular epithelial cells.
Pediatric research.
2022 04; 91(5):1121-1129. doi:
10.1038/s41390-021-01622-3. [PMID: 34155339] - Pramod K Mistry, Elena Lukina, Hadhami Ben Turkia, Suma P Shankar, Hagit Baris Feldman, Marwan Ghosn, Atul Mehta, Seymour Packman, Heather Lau, Milan Petakov, Sarit Assouline, Manisha Balwani, Sumita Danda, Evgueniy Hadjiev, Andres Ortega, Meredith C Foster, Sebastiaan J M Gaemers, M Judith Peterschmitt. Clinical outcomes after 4.5 years of eliglustat therapy for Gaucher disease type 1: Phase 3 ENGAGE trial final results.
American journal of hematology.
2021 09; 96(9):1156-1165. doi:
10.1002/ajh.26276. [PMID: 34161616] - Jody Vykoukal, Johannes F Fahrmann, Justin R Gregg, Zhe Tang, Spyridon Basourakos, Ehsan Irajizad, Sanghee Park, Guang Yang, Chad J Creighton, Alia Fleury, Jeffrey Mayo, Adriana Paulucci-Holthauzen, Jennifer B Dennison, Eunice Murage, Christine B Peterson, John W Davis, Jeri Kim, Samir Hanash, Timothy C Thompson. Caveolin-1-mediated sphingolipid oncometabolism underlies a metabolic vulnerability of prostate cancer.
Nature communications.
2020 08; 11(1):4279. doi:
10.1038/s41467-020-17645-z. [PMID: 32855410] - Shiny Nair, Noffar Bar, Mina L Xu, Madhav Dhodapkar, Pramod K Mistry. Glucosylsphingosine but not Saposin C, is the target antigen in Gaucher disease-associated gammopathy.
Molecular genetics and metabolism.
2020 04; 129(4):286-291. doi:
10.1016/j.ymgme.2020.01.009. [PMID: 32044242] - Jing Li, Jun Chen, Vanaja Kanamaluru, Sebastiaan J M Gaemers, M Judith Peterschmitt, Audrey W Hou, Yong Xue, Sandrine Turpault, Dan Rudin. Impact of hepatic and renal impairment on the pharmacokinetics and tolerability of eliglustat therapy for Gaucher disease type 1.
Molecular genetics and metabolism.
2020 02; 129(2):117-124. doi:
10.1016/j.ymgme.2019.11.002. [PMID: 31924461] - Jingjing Chen, Yuanyuan Shao, Huidan Zhu, Xiufang Chen, Xuemei Ye. Liquid chromatography-tandem mass spectrometric method for the quantification of eliglustat in rat plasma and the application in a pre-clinical study.
Journal of pharmaceutical and biomedical analysis.
2020 Jan; 177(?):112858. doi:
10.1016/j.jpba.2019.112858. [PMID: 31518862] - Elena V Pavlova, Aleksey Shatunov, Lena Wartosch, Alena I Moskvina, Lena E Nikolaeva, Nicholas A Bright, Karen L Tylee, Heather J Church, Andrea Ballabio, J Paul Luzio, Timothy M Cox. The lysosomal disease caused by mutant VPS33A.
Human molecular genetics.
2019 08; 28(15):2514-2530. doi:
10.1093/hmg/ddz077. [PMID: 31070736] - Venkata Krishna Reddy, Narayana Swamy, Rajeswari Rathod, Pinaki Sengupta. A Bioanalytical Method for Eliglustat Quantification in Rat Plasma.
Journal of chromatographic science.
2019 Aug; 57(7):600-605. doi:
10.1093/chromsci/bmz033. [PMID: 31095671] - Qiong Wang, Haiyun Wang, Youyan Zhong, Qiang Zhang. Drug-Drug Interactions Of Amiodarone And Quinidine On The Pharmacokinetics Of Eliglustat In Rats.
Drug design, development and therapy.
2019; 13(?):4207-4213. doi:
10.2147/dddt.s226948. [PMID: 31849452] - Elena Lukina, Nora Watman, Marta Dragosky, Heather Lau, Elsa Avila Arreguin, Hanna Rosenbaum, Ari Zimran, Meredith C Foster, Sebastiaan J M Gaemers, M Judith Peterschmitt. Outcomes after 8 years of eliglustat therapy for Gaucher disease type 1: Final results from the Phase 2 trial.
American journal of hematology.
2019 01; 94(1):29-38. doi:
10.1002/ajh.25300. [PMID: 30264864] - Justin J Kang, Nayiri M Kaissarian, Karl C Desch, Robert J Kelly, Liming Shu, Peter F Bodary, James A Shayman. α-galactosidase A deficiency promotes von Willebrand factor secretion in models of Fabry disease.
Kidney international.
2019 01; 95(1):149-159. doi:
10.1016/j.kint.2018.08.033. [PMID: 30470436] - Pramod K Mistry, Elena Lukina, Hadhami Ben Turkia, Suma P Shankar, Hagit Baris, Marwan Ghosn, Atul Mehta, Seymour Packman, Gregory Pastores, Milan Petakov, Sarit Assouline, Manisha Balwani, Sumita Danda, Evgueniy Hadjiev, Andres Ortega, Sebastiaan J M Gaemers, Regina Tayag, M Judith Peterschmitt. Outcomes after 18 months of eliglustat therapy in treatment-naïve adults with Gaucher disease type 1: The phase 3 ENGAGE trial.
American journal of hematology.
2017 Nov; 92(11):1170-1176. doi:
10.1002/ajh.24877. [PMID: 28762527] - Vagishwari Murugesan, Wei-Lien Chuang, Jun Liu, Andrew Lischuk, Katherine Kacena, Haiqun Lin, Gregory M Pastores, Ruhua Yang, Joan Keutzer, Kate Zhang, Pramod K Mistry. Glucosylsphingosine is a key biomarker of Gaucher disease.
American journal of hematology.
2016 Nov; 91(11):1082-1089. doi:
10.1002/ajh.24491. [PMID: 27441734] - Bouwien E Smid, Maria J Ferraz, Marri Verhoek, Mina Mirzaian, Patrick Wisse, Herman S Overkleeft, Carla E Hollak, Johannes M Aerts. Biochemical response to substrate reduction therapy versus enzyme replacement therapy in Gaucher disease type 1 patients.
Orphanet journal of rare diseases.
2016 Mar; 11(?):28. doi:
10.1186/s13023-016-0413-3. [PMID: 27008851] - Elena V Pavlova, Joy Archer, Susan Wang, Nick Dekker, Johannes Mfg Aerts, Stefan Karlsson, Timothy M Cox. Inhibition of UDP-glucosylceramide synthase in mice prevents Gaucher disease-associated B-cell malignancy.
The Journal of pathology.
2015 Jan; 235(1):113-24. doi:
10.1002/path.4452. [PMID: 25256118] - Lunawati L Bennett, Kelsey Turcotte. Eliglustat tartrate for the treatment of adults with type 1 Gaucher disease.
Drug design, development and therapy.
2015; 9(?):4639-47. doi:
10.2147/dddt.s77760. [PMID: 26345314] - Elena Lukina, Nora Watman, Marta Dragosky, Gregory M Pastores, Elsa Avila Arreguin, Hanna Rosenbaum, Ari Zimran, Jennifer Angell, Leorah Ross, Ana Cristina Puga, Judith M Peterschmitt. Eliglustat, an investigational oral therapy for Gaucher disease type 1: Phase 2 trial results after 4 years of treatment.
Blood cells, molecules & diseases.
2014 Dec; 53(4):274-6. doi:
10.1016/j.bcmd.2014.04.002. [PMID: 24835462] - Raewyn M Poole. Eliglustat: first global approval.
Drugs.
2014 Oct; 74(15):1829-36. doi:
10.1007/s40265-014-0296-3. [PMID: 25239269] - Lunawati L Bennett, Devipriya Mohan. Gaucher disease and its treatment options.
The Annals of pharmacotherapy.
2013 Sep; 47(9):1182-93. doi:
10.1177/1060028013500469. [PMID: 24259734] - James A Shayman. The design and clinical development of inhibitors of glycosphingolipid synthesis: will invention be the mother of necessity?.
Transactions of the American Clinical and Climatological Association.
2013; 124(?):46-60. doi:
NULL. [PMID: 23874009] - M Judith Peterschmitt, Amy Burke, Larry Blankstein, Sharon E Smith, Ana Cristina Puga, William G Kramer, James A Harris, David Mathews, Peter L Bonate. Safety, tolerability, and pharmacokinetics of eliglustat tartrate (Genz-112638) after single doses, multiple doses, and food in healthy volunteers.
Journal of clinical pharmacology.
2011 May; 51(5):695-705. doi:
10.1177/0091270010372387. [PMID: 20864621] - John Marshall, Karen M Ashe, Dinesh Bangari, KerryAnne McEachern, Wei-Lien Chuang, Joshua Pacheco, Diane P Copeland, Robert J Desnick, James A Shayman, Ronald K Scheule, Seng H Cheng. Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease.
PloS one.
2010 Nov; 5(11):e15033. doi:
10.1371/journal.pone.0015033. [PMID: 21124789] - Timothy M Cox. Eliglustat tartrate, an orally active glucocerebroside synthase inhibitor for the potential treatment of Gaucher disease and other lysosomal storage diseases.
Current opinion in investigational drugs (London, England : 2000).
2010 Oct; 11(10):1169-81. doi:
NULL. [PMID: 20872320] - Elena Lukina, Nora Watman, Elsa Avila Arreguin, Maryam Banikazemi, Marta Dragosky, Marcelo Iastrebner, Hanna Rosenbaum, Mici Phillips, Gregory M Pastores, Daniel I Rosenthal, Mathilde Kaper, Tejdip Singh, Ana Cristina Puga, Peter L Bonate, M Judith Peterschmitt. A phase 2 study of eliglustat tartrate (Genz-112638), an oral substrate reduction therapy for Gaucher disease type 1.
Blood.
2010 Aug; 116(6):893-9. doi:
10.1182/blood-2010-03-273151. [PMID: 20439622]