Glucosylsphingosine (BioDeep_00001869553)
Main id: BioDeep_00000014356
PANOMIX_OTCML-2023 Volatile Flavor Compounds
代谢物信息卡片
Glucosylsphingosine
化学式: C24H47NO7 (461.3352352)
中文名称: D-葡萄糖基-β1-1'-D-赤型-鞘氨醇
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)O)O)O)N)O
InChI: InChI=1S/C24H47NO7/c1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-19(27)18(25)17-31-24-23(30)22(29)21(28)20(16-26)32-24/h14-15,18-24,26-30H,2-13,16-17,25H2,1H3/b15-14+/t18-,19+,20+,21+,22-,23+,24+/m0/s1
描述信息
Glucosylsphingosine (lyso-Gb1) is a deacylated form of glucosylceramide and is also degraded by the glucocerebrosidase. Glucosylsphingosine is a very promising, reliable and specific biomarker for monitoring Gaucher disease[1].
同义名列表
数据库引用编号
8 个数据库交叉引用编号
- ChEBI: CHEBI:71981
- KEGG: C03108
- PubChem: 5280570
- CAS: 52050-17-6
- ChEBI: CHEBI:185325
- PubChem: 6004
- NIKKAJI: J675.987D
- medchemexpress: HY-N7745
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)
1 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
- Makaila L Furderer, Bahafta Berhe, Tiffany C Chen, Stephen Wincovitch, Xuntian Jiang, Nahid Tayebi, Ellen Sidransky, Tae-Un Han. A Comparative Biochemical and Pathological Evaluation of Brain Samples from Knock-In Murine Models of Gaucher Disease.
International journal of molecular sciences.
2024 Feb; 25(3):. doi:
10.3390/ijms25031827. [PMID: 38339105] - 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] - Casey L Mahoney-Crane, Megha Viswanathan, Dreson Russell, Rachel A C Curtiss, Jennifer Freire, Sai Sumedha Bobba, Sean D Coyle, Monika Kandebo, Lihang Yao, Bang-Lin Wan, Nathan G Hatcher, Sean M Smith, Jacob N Marcus, Laura A Volpicelli-Daley. Neuronopathic GBA1L444P Mutation Accelerates Glucosylsphingosine Levels and Formation of Hippocampal Alpha-Synuclein Inclusions.
The Journal of neuroscience : the official journal of the Society for Neuroscience.
2023 01; 43(3):501-521. doi:
10.1523/jneurosci.0680-22.2022. [PMID: 36639889] - Shin-Ichi Matsumoto, Sho Sato, Kentaro Otake, Yohei Kosugi. Highly-sensitive simultaneous quantitation of glucosylsphingosine and galactosylsphingosine in human cerebrospinal fluid by liquid chromatography/tandem mass spectrometry.
Journal of pharmaceutical and biomedical analysis.
2022 Aug; 217(?):114852. doi:
10.1016/j.jpba.2022.114852. [PMID: 35636011] - Lindsey T Lelieveld, Sophie Gerhardt, Saskia Maas, Kimberley C Zwiers, Claire de Wit, Ernst H Beijk, Maria J Ferraz, Marta Artola, Annemarie H Meijer, Christian Tudorache, Daniela Salvatori, Rolf G Boot, Johannes M F G Aerts. Consequences of excessive glucosylsphingosine in glucocerebrosidase-deficient zebrafish.
Journal of lipid research.
2022 05; 63(5):100199. doi:
10.1016/j.jlr.2022.100199. [PMID: 35315333] - Matthew Surface, Manisha Balwani, Cheryl Waters, Alexander Haimovich, Ziv Gan-Or, Karen S Marder, Tammy Hsieh, Linxia Song, Shalini Padmanabhan, Frank Hsieh, Kalpana M Merchant, Roy N Alcalay. Plasma Glucosylsphingosine in GBA1 Mutation Carriers with and without Parkinson's Disease.
Movement disorders : official journal of the Movement Disorder Society.
2022 02; 37(2):416-421. doi:
10.1002/mds.28846. [PMID: 34741486] - Manasa P Srikanth, Jace W Jones, Maureen Kane, Ola Awad, Tea Soon Park, Elias T Zambidis, Ricardo A Feldman. Elevated glucosylsphingosine in Gaucher disease induced pluripotent stem cell neurons deregulates lysosomal compartment through mammalian target of rapamycin complex 1.
Stem cells translational medicine.
2021 07; 10(7):1081-1094. doi:
10.1002/sctm.20-0386. [PMID: 33656802] - James Beasley, Patricia McCaw, Haoyue Zhang, Sarah P Young, Ashlee R Stiles. Combined analysis of plasma or serum glucosylsphingosine and globotriaosylsphingosine by UPLC-MS/MS.
Clinica chimica acta; international journal of clinical chemistry.
2020 Dec; 511(?):132-137. doi:
10.1016/j.cca.2020.10.007. [PMID: 33058838] - X Zhan, H W Zhang, X L Gao, X F Gu, L J Chen, R B Guo. [Application of plasma glucosylsphingosine detection in the follow-up of patients with Gaucher disease].
Zhonghua yi xue za zhi.
2020 Nov; 100(40):3169-3173. doi:
10.3760/cma.j.cn112137-20200717-02138. [PMID: 33142401] - Iskren Menkovic, Michel Boutin, Abdulfatah Alayoubi, François E Mercier, Georges-Étienne Rivard, Christiane Auray-Blais. Identification of a Reliable Biomarker Profile for the Diagnosis of Gaucher Disease Type 1 Patients Using a Mass Spectrometry-Based Metabolomic Approach.
International journal of molecular sciences.
2020 Oct; 21(21):. doi:
10.3390/ijms21217869. [PMID: 33114153] - Shoshana Revel-Vilk, Maria Fuller, Ari Zimran. Value of Glucosylsphingosine (Lyso-Gb1) as a Biomarker in Gaucher Disease: A Systematic Literature Review.
International journal of molecular sciences.
2020 Sep; 21(19):. doi:
10.3390/ijms21197159. [PMID: 32998334] - Lucie Dupuis, Caroline Chipeaux, Emmanuelle Bourdelier, Suella Martino, Nelly Reihani, Nadia Belmatoug, Thierry Billette de Villemeur, Bénédicte Hivert, Fathi Moussa, Caroline Le Van Kim, Marine de Person, Mélanie Franco. Effects of sphingolipids overload on red blood cell properties in Gaucher disease.
Journal of cellular and molecular medicine.
2020 09; 24(17):9726-9736. doi:
10.1111/jcmm.15534. [PMID: 32767726] - Shi-En Chen, Shuyun Zhu, Jingwen Hu, Jing Sun, Zhenjia Zheng, Xian-En Zhao, Huwei Liu. 8-Plex stable isotope labeling absolute quantitation strategy combined with dual-targeted recognizing function material for simultaneous separation and determination of glucosylsphingosine and galactosylsphingosine in human plasma.
Analytica chimica acta.
2020 Aug; 1124(?):40-51. doi:
10.1016/j.aca.2020.05.032. [PMID: 32534674] - Jennifer T Saville, Belinda K McDermott, Sharon J Chin, Janice M Fletcher, Maria Fuller. Expanding the clinical utility of glucosylsphingosine for Gaucher disease.
Journal of inherited metabolic disease.
2020 05; 43(3):558-563. doi:
10.1002/jimd.12192. [PMID: 31707742] - Pilar Irún, Jorge J Cebolla, Laura López de Frutos, Isabel De Castro-Orós, Mercedes Roca-Espiau, Pilar Giraldo. LC-MS/MS analysis of plasma glucosylsphingosine as a biomarker for diagnosis and follow-up monitoring in Gaucher disease in the Spanish population.
Clinical chemistry and laboratory medicine.
2020 04; 58(5):798-809. doi:
10.1515/cclm-2019-0949. [PMID: 32126008] - Simona Di Martino, Piero Tardia, Vincenzo Cilibrasi, Samantha Caputo, Marco Mazzonna, Debora Russo, Ilaria Penna, Natalia Realini, Natasha Margaroli, Marco Migliore, Daniela Pizzirani, Giuliana Ottonello, Sine Mandrup Bertozzi, Andrea Armirotti, Duc Nguyen, Ying Sun, Ernesto R Bongarzone, Peter Lansbury, Min Liu, Renato Skerlj, Rita Scarpelli. Lead Optimization of Benzoxazolone Carboxamides as Orally Bioavailable and CNS Penetrant Acid Ceramidase Inhibitors.
Journal of medicinal chemistry.
2020 04; 63(7):3634-3664. doi:
10.1021/acs.jmedchem.9b02004. [PMID: 32176488] - 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] - Wei-Lien Chuang, Joshua Pacheco, Dritan Hoxha, Gerard Sanderink, Crystal Sung. Galactosylsphingosine does not interfere with the quantitation of plasma glucosylsphingosine levels in Gaucher patients.
Clinica chimica acta; international journal of clinical chemistry.
2019 Jul; 494(?):48-51. doi:
10.1016/j.cca.2019.03.009. [PMID: 30858093] - Muhammad Zain Chauhan, Ann-Katrin Valencia, Maria Carmen Piqueras, Mabel Enriquez-Algeciras, Sanjoy K Bhattacharya. Optic Nerve Lipidomics Reveal Impaired Glucosylsphingosine Lipids Pathway in Glaucoma.
Investigative ophthalmology & visual science.
2019 04; 60(5):1789-1798. doi:
10.1167/iovs.18-25802. [PMID: 31022733] - Rohini Sidhu, Christina R Mikulka, Hideji Fujiwara, Mark S Sands, Jean E Schaffer, Daniel S Ory, Xuntian Jiang. A HILIC-MS/MS method for simultaneous quantification of the lysosomal disease markers galactosylsphingosine and glucosylsphingosine in mouse serum.
Biomedical chromatography : BMC.
2018 Jul; 32(7):e4235. doi:
10.1002/bmc.4235. [PMID: 29516569] - Anna Tylki-Szymańska, Paulina Szymańska-Rożek, Piotr Hasiński, Agnieszka Ługowska. Plasma chitotriosidase activity versus plasma glucosylsphingosine in wide spectrum of Gaucher disease phenotypes - A statistical insight.
Molecular genetics and metabolism.
2018 04; 123(4):495-500. doi:
10.1016/j.ymgme.2018.02.004. [PMID: 29530534] - Lulu Kang, Xia Zhan, Xuefan Gu, Huiwen Zhang. Successful newborn screening for Gaucher disease using fluorometric assay in China.
Journal of human genetics.
2017 Aug; 62(8):763-768. doi:
10.1038/jhg.2017.36. [PMID: 28356566] - 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] - Paul A Gramlich, Wendy Westbroek, Ricardo A Feldman, Ola Awad, Nicholas Mello, Mary P Remington, Ying Sun, Wujuan Zhang, Ellen Sidransky, Michael J Betenbaugh, Paul S Fishman. A peptide-linked recombinant glucocerebrosidase for targeted neuronal delivery: Design, production, and assessment.
Journal of biotechnology.
2016 Mar; 221(?):1-12. doi:
10.1016/j.jbiotec.2016.01.015. [PMID: 26795355] - Maria Fuller, Jeff Szer, Samantha Stark, Janice M Fletcher. Rapid, single-phase extraction of glucosylsphingosine from plasma: A universal screening and monitoring tool.
Clinica chimica acta; international journal of clinical chemistry.
2015 Oct; 450(?):6-10. doi:
10.1016/j.cca.2015.07.026. [PMID: 26232157] - Mina Mirzaian, Patrick Wisse, Maria J Ferraz, Henrik Gold, Wilma E Donker-Koopman, Marri Verhoek, Herman S Overkleeft, Rolf G Boot, Gertjan Kramer, Nick Dekker, Johannes M F G Aerts. Mass spectrometric quantification of glucosylsphingosine in plasma and urine of type 1 Gaucher patients using an isotope standard.
Blood cells, molecules & diseases.
2015 Apr; 54(4):307-14. doi:
10.1016/j.bcmd.2015.01.006. [PMID: 25842368] - Ying Sun, Jane Florer, Christopher N Mayhew, Zhanfeng Jia, Zhiying Zhao, Kui Xu, Huimin Ran, Benjamin Liou, Wujuan Zhang, Kenneth D R Setchell, Jianguo Gu, Gregory A Grabowski. Properties of neurons derived from induced pluripotent stem cells of Gaucher disease type 2 patient fibroblasts: potential role in neuropathology.
PloS one.
2015; 10(3):e0118771. doi:
10.1371/journal.pone.0118771. [PMID: 25822147] - Richard W D Welford, Marco Garzotti, Charles Marques Lourenço, Eugen Mengel, Thorsten Marquardt, Janine Reunert, Yasmina Amraoui, Stefan A Kolb, Olivier Morand, Peter Groenen. Plasma lysosphingomyelin demonstrates great potential as a diagnostic biomarker for Niemann-Pick disease type C in a retrospective study.
PloS one.
2014; 9(12):e114669. doi:
10.1371/journal.pone.0114669. [PMID: 25479233] - Paulo Gaspar, Wouter W Kallemeijn, Anneke Strijland, Saskia Scheij, Marco Van Eijk, Jan Aten, Herman S Overkleeft, Andrea Balreira, Friederike Zunke, Michael Schwake, Clara Sá Miranda, Johannes M F G Aerts. Action myoclonus-renal failure syndrome: diagnostic applications of activity-based probes and lipid analysis.
Journal of lipid research.
2014 Jan; 55(1):138-45. doi:
10.1194/jlr.m043802. [PMID: 24212238] - E V Pavlova, S Z Wang, J Archer, N Dekker, J M F G Aerts, S Karlsson, T M Cox. B cell lymphoma and myeloma in murine Gaucher's disease.
The Journal of pathology.
2013 Sep; 231(1):88-97. doi:
10.1002/path.4227. [PMID: 23775597] - S Pablo Sardi, Jennifer Clarke, Catherine Viel, Monyrath Chan, Thomas J Tamsett, Christopher M Treleaven, Jie Bu, Lindsay Sweet, Marco A Passini, James C Dodge, W Haung Yu, Richard L Sidman, Seng H Cheng, Lamya S Shihabuddin. Augmenting CNS glucocerebrosidase activity as a therapeutic strategy for parkinsonism and other Gaucher-related synucleinopathies.
Proceedings of the National Academy of Sciences of the United States of America.
2013 Feb; 110(9):3537-42. doi:
10.1073/pnas.1220464110. [PMID: 23297226] - Ying Sun, Benjamin Liou, You-Hai Xu, Brian Quinn, Wujuan Zhang, Rick Hamler, Kenneth D R Setchell, Gregory A Grabowski. Ex vivo and in vivo effects of isofagomine on acid β-glucosidase variants and substrate levels in Gaucher disease.
The Journal of biological chemistry.
2012 Feb; 287(6):4275-87. doi:
10.1074/jbc.m111.280016. [PMID: 22167193] - Nick Dekker, Laura van Dussen, Carla E M Hollak, Herman Overkleeft, Saskia Scheij, Karen Ghauharali, Mariëlle J van Breemen, Maria J Ferraz, Johanna E M Groener, Mario Maas, Frits A Wijburg, Dave Speijer, Anna Tylki-Szymanska, Pramod K Mistry, Rolf G Boot, Johannes M Aerts. Elevated plasma glucosylsphingosine in Gaucher disease: relation to phenotype, storage cell markers, and therapeutic response.
Blood.
2011 Oct; 118(16):e118-27. doi:
10.1182/blood-2011-05-352971. [PMID: 21868580] - Christiane Auray-Blais, Aimé Ntwari, Joe T R Clarke, David G Warnock, João Paulo Oliveira, Sarah P Young, David S Millington, Daniel G Bichet, Sandra Sirrs, Michael L West, Robin Casey, Wuh-Liang Hwu, Joan M Keutzer, X Kate Zhang, René Gagnon. How well does urinary lyso-Gb3 function as a biomarker in Fabry disease?.
Clinica chimica acta; international journal of clinical chemistry.
2010 Dec; 411(23-24):1906-14. doi:
10.1016/j.cca.2010.07.038. [PMID: 20716442] - Hiroshi Deguchi, Subramanian Yegneswaran, John H Griffin. Sphingolipids as bioactive regulators of thrombin generation.
The Journal of biological chemistry.
2004 Mar; 279(13):12036-42. doi:
10.1074/jbc.m302531200. [PMID: 14722105] - Jacques Bodennec, Selena Trajkovic-Bodennec, Anthony H Futerman. Simultaneous quantification of lyso-neutral glycosphingolipids and neutral glycosphingolipids by N-acetylation with [3H]acetic anhydride.
Journal of lipid research.
2003 Jul; 44(7):1413-9. doi:
10.1194/jlr.d300010-jlr200. [PMID: 12730305] - Reiko Okamoto, Junko Arikawa, Mutsumi Ishibashi, Makoto Kawashima, Yutaka Takagi, Genji Imokawa. Sphingosylphosphorylcholine is upregulated in the stratum corneum of patients with atopic dermatitis.
Journal of lipid research.
2003 Jan; 44(1):93-102. doi:
10.1194/jlr.m200225-jlr200. [PMID: 12518027] - Dan J Sillence, Vishwajeet Puri, David L Marks, Terry D Butters, Raymond A Dwek, Richard E Pagano, Frances M Platt. Glucosylceramide modulates membrane traffic along the endocytic pathway.
Journal of lipid research.
2002 Nov; 43(11):1837-45. doi:
10.1194/jlr.m200232-jlr200. [PMID: 12401882] - Eduard Orvisky, Joseph K Park, Mary E LaMarca, Edward I Ginns, Brian M Martin, Nahid Tayebi, Ellen Sidransky. Glucosylsphingosine accumulation in tissues from patients with Gaucher disease: correlation with phenotype and genotype.
Molecular genetics and metabolism.
2002 Aug; 76(4):262-70. doi:
10.1016/s1096-7192(02)00117-8. [PMID: 12208131] - H Sprong, S Degroote, T Claessens, J van Drunen, V Oorschot, B H Westerink, Y Hirabayashi, J Klumperman, P van der Sluijs, G van Meer. Glycosphingolipids are required for sorting melanosomal proteins in the Golgi complex.
The Journal of cell biology.
2001 Oct; 155(3):369-80. doi:
10.1083/jcb.200106104. [PMID: 11673476] - N Sueyoshi, T Maehara, M Ito. Apoptosis of Neuro2a cells induced by lysosphingolipids with naturally occurring stereochemical configurations.
Journal of lipid research.
2001 Aug; 42(8):1197-202. doi:
10.1016/s0022-2275(20)31569-8. [PMID: 11483620] - A Bischoff, D Meyer Zu Heringdorf, K H Jakobs, M C Michel. Lysosphingolipid receptor-mediated diuresis and natriuresis in anaesthetized rats.
British journal of pharmacology.
2001 Apr; 132(8):1925-33. doi:
10.1038/sj.bjp.0703969. [PMID: 11309265] - A Bischoff, P Czyborra, D Meyer Zu Heringdorf, K H Jakobs, M C Michel. Sphingosine-1-phosphate reduces rat renal and mesenteric blood flow in vivo in a pertussis toxin-sensitive manner.
British journal of pharmacology.
2000 Aug; 130(8):1878-83. doi:
10.1038/sj.bjp.0703516. [PMID: 10952678] - T Kanazawa, S Nakamura, M Momoi, T Yamaji, H Takematsu, H Yano, H Sabe, A Yamamoto, T Kawasaki, Y Kozutsumi. Inhibition of cytokinesis by a lipid metabolite, psychosine.
The Journal of cell biology.
2000 May; 149(4):943-50. doi:
10.1083/jcb.149.4.943. [PMID: 10811833] - M Nozawa, T Iwamoto, T Tokoro, Y Eto. Novel procedure for measuring psychosine derivatives by an HPLC method.
Journal of neurochemistry.
1992 Aug; 59(2):607-9. doi:
10.1111/j.1471-4159.1992.tb09412.x. [PMID: 1629732] - H Igisu, N Hamasaki, A Ito, W Ou. Inhibition of cytochrome c oxidase and hemolysis caused by lysosphingolipids.
Lipids.
1988 Apr; 23(4):345-8. doi:
10.1007/bf02537346. [PMID: 2840545] - S Gatt, T Dinur, K Osiecki, R J Desnick, G A Grabowski. Use of activators and inhibitors to define the properties of the active site of normal and Gaucher disease lysosomal beta-glucosidase.
Enzyme.
1985; 33(2):109-19. doi:
10.1159/000469416. [PMID: 3924590] - S S Raghavan, R A Mumford, J N Kanfer. Isolation and characterization of glucosylsphingosine from Gaucher's spleen.
Journal of lipid research.
1974 Sep; 15(5):484-90. doi:
10.1016/s0022-2275(20)36768-7. [PMID: 4414001]