Psychosine (BioDeep_00000270846)

Main id: BioDeep_00000001415

 

natural product


代谢物信息卡片


1-beta-galactosyl-sphing-4-enine

化学式: C24H47NO7 (461.3352352)
中文名称: D-半乳糖基-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: CCCCCCCCCCCCC/C=C/[C@@H](O)[C@@H](N)CO[C@@H]1O[C@H](CO)[C@H](O)[C@H](O)[C@H]1O
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

A glycosylsphingoid consisting of sphingosine having a beta-D-galactosyl residue attached at the 1-position.

同义名列表

5 个代谢物同义名

Psychosine; 1-beta-galactosyl-sphing-4-enine; Galactosylsphingosine; Glucosyl-C18-sphingosine; Psychosine



数据库引用编号

16 个数据库交叉引用编号

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)

7 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]
  • Husam B R Alabed, Ambra Del Grosso, Valeria Bellani, Lorena Urbanelli, Sara Carpi, Miriam De Sarlo, Lorenzo Bertocci, Laura Colagiorgio, Sandra Buratta, Luca Scaccini, Dorotea Frongia Mancini, Ilaria Tonazzini, Marco Cecchini, Carla Emiliani, Roberto Maria Pellegrino. Untargeted Lipidomic Approach for Studying Different Nervous System Tissues of the Murine Model of Krabbe Disease. Biomolecules. 2023 10; 13(10):. doi: 10.3390/biom13101562. [PMID: 37892244]
  • Takashi Watanabe, Kazuhito Tsuboi, Nobuaki Matsuda, Yuta Ishizuka, Shinji Go, Etsuko Watanabe, Ayaka Ono, Yasuo Okamoto, Junko Matsuda. Genetic ablation of Saposin-D in Krabbe disease eliminates psychosine accumulation but does not significantly improve demyelination. Journal of neurochemistry. 2023 Jun; ?(?):. doi: 10.1111/jnc.15876. [PMID: 37337846]
  • 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]
  • Elisabeth Mangiameli, Anna Cecchele, Francesco Morena, Francesca Sanvito, Vittoria Matafora, Angela Cattaneo, Lucrezia Della Volpe, Daniela Gnani, Marianna Paulis, Lucia Susani, Sabata Martino, Raffaella Di Micco, Angela Bachi, Angela Gritti. Human iPSC-based neurodevelopmental models of globoid cell leukodystrophy uncover patient- and cell type-specific disease phenotypes. Stem cell reports. 2021 06; 16(6):1478-1495. doi: 10.1016/j.stemcr.2021.04.011. [PMID: 33989519]
  • 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]
  • S Gowrishankar, S M Cologna, M I Givogri, E R Bongarzone. Deregulation of signalling in genetic conditions affecting the lysosomal metabolism of cholesterol and galactosyl-sphingolipids. Neurobiology of disease. 2020 12; 146(?):105142. doi: 10.1016/j.nbd.2020.105142. [PMID: 33080336]
  • 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]
  • Carley R Corado, Jason Pinkstaff, Xuntian Jiang, Evelyn M Galban, Samantha J Fisher, Oriane Scholler, Chris Russell, Jessica H Bagel, Patricia A ODonnell, Daniel S Ory, Charles H Vite, Allison M Bradbury. Cerebrospinal fluid and serum glycosphingolipid biomarkers in canine globoid cell leukodystrophy (Krabbe Disease). Molecular and cellular neurosciences. 2020 01; 102(?):103451. doi: 10.1016/j.mcn.2019.103451. [PMID: 31794880]
  • 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]
  • Tuba Sural-Fehr, Harinder Singh, Ludovico Cantuti-Catelvetri, Hongling Zhu, Michael S Marshall, Rima Rebiai, Martin J Jastrzebski, Maria I Givogri, Mark M Rasenick, Ernesto R Bongarzone. Inhibition of the IGF-1-PI3K-Akt-mTORC2 pathway in lipid rafts increases neuronal vulnerability in a genetic lysosomal glycosphingolipidosis. Disease models & mechanisms. 2019 05; 12(5):. doi: 10.1242/dmm.036590. [PMID: 31036560]
  • 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]
  • Yenisleidy de Las Mercedes Zulueta Díaz, Sofia Caby, Ernesto R Bongarzone, María Laura Fanani. Psychosine remodels model lipid membranes at neutral pH. Biochimica et biophysica acta. Biomembranes. 2018 12; 1860(12):2515-2526. doi: 10.1016/j.bbamem.2018.09.015. [PMID: 30267657]
  • 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]
  • S Zhao, X Zhan, Y Wang, J Ye, L Han, W Qiu, X Gao, X Gu, H Zhang. Large-scale study of clinical and biochemical characteristics of Chinese patients diagnosed with Krabbe disease. Clinical genetics. 2018 02; 93(2):248-254. doi: 10.1111/cge.13071. [PMID: 28598007]
  • 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]
  • Ludovic D'Auria, Cory Reiter, Emma Ward, Ana Lis Moyano, Michael S Marshall, Duc Nguyen, Giuseppe Scesa, Zane Hauck, Richard van Breemen, Maria I Givogri, Ernesto R Bongarzone. Psychosine enhances the shedding of membrane microvesicles: Implications in demyelination in Krabbe's disease. PloS one. 2017; 12(5):e0178103. doi: 10.1371/journal.pone.0178103. [PMID: 28531236]
  • Cedric Misslin, Maria Velasco-Estevez, Marie Albert, Sinead A O'Sullivan, Kumlesh K Dev. Phospholipase A2 is involved in galactosylsphingosine-induced astrocyte toxicity, neuronal damage and demyelination. PloS one. 2017; 12(11):e0187217. doi: 10.1371/journal.pone.0187217. [PMID: 29095858]
  • Stefka Spassieva, Erhard Bieberich. Lysosphingolipids and sphingolipidoses: Psychosine in Krabbe's disease. Journal of neuroscience research. 2016 11; 94(11):974-81. doi: 10.1002/jnr.23888. [PMID: 27638582]
  • Joana Nogueira-Rodrigues, Pedro Brites, Mónica Mendes Sousa. Axonal pathology in Krabbe's disease: The cytoskeleton as an emerging therapeutic target. Journal of neuroscience research. 2016 11; 94(11):1037-41. doi: 10.1002/jnr.23771. [PMID: 27638589]
  • 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]
  • Jacqueline A Hawkins-Salsbury, Lauren Shea, Xuntian Jiang, Daniel A Hunter, A Miguel Guzman, Adarsh S Reddy, Elizabeth Y Qin, Yedda Li, Steven J Gray, Daniel S Ory, Mark S Sands. Mechanism-based combination treatment dramatically increases therapeutic efficacy in murine globoid cell leukodystrophy. The Journal of neuroscience : the official journal of the Society for Neuroscience. 2015 Apr; 35(16):6495-505. doi: 10.1523/jneurosci.4199-14.2015. [PMID: 25904800]
  • Mina Mirzaian, Gertjan Kramer, Ben J H M Poorthuis. Quantification of sulfatides and lysosulfatides in tissues and body fluids by liquid chromatography-tandem mass spectrometry. Journal of lipid research. 2015 Apr; 56(4):936-43. doi: 10.1194/jlr.m057232. [PMID: 25632048]
  • 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]
  • Subramanian Yegneswaran, Yajnavalka Banerjee, José A Fernández, Hiroshi Deguchi, John H Griffin. Lyso-Sulfatide Binds Factor Xa and Inhibits Thrombin Generation by the Prothrombinase Complex. PloS one. 2015; 10(8):e0135025. doi: 10.1371/journal.pone.0135025. [PMID: 26263376]
  • 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]
  • Tamar Farfel-Becker, Einat B Vitner, Samuel L Kelly, Jessica R Bame, Jingjing Duan, Vera Shinder, Alfred H Merrill, Kostantin Dobrenis, Anthony H Futerman. Neuronal accumulation of glucosylceramide in a mouse model of neuronopathic Gaucher disease leads to neurodegeneration. Human molecular genetics. 2014 Feb; 23(4):843-54. doi: 10.1093/hmg/ddt468. [PMID: 24064337]
  • 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]
  • Jacqueline A Hawkins-Salsbury, Archana R Parameswar, Xuntian Jiang, Paul H Schlesinger, Ernesto Bongarzone, Daniel S Ory, Alexei V Demchenko, Mark S Sands. Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture. Journal of lipid research. 2013 Dec; 54(12):3303-11. doi: 10.1194/jlr.m039610. [PMID: 24006512]
  • Katyayini Aribindi, Yenifer Guerra, Maria Del Carmen Piqueras, James T Banta, Richard K Lee, Sanjoy K Bhattacharya. Cholesterol and glycosphingolipids of human trabecular meshwork and aqueous humor: comparative profiles from control and glaucomatous donors. Current eye research. 2013 Oct; 38(10):1017-26. doi: 10.3109/02713683.2013.803123. [PMID: 23790057]
  • 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]
  • Ludovico Cantuti Castelvetri, Maria I Givogri, Amy Hebert, Benjamin Smith, Yuyu Song, Agnieszka Kaminska, Aurora Lopez-Rosas, Gerardo Morfini, Gustavo Pigino, Mark Sands, Scott T Brady, Ernesto R Bongarzone. The sphingolipid psychosine inhibits fast axonal transport in Krabbe disease by activation of GSK3β and deregulation of molecular motors. The Journal of neuroscience : the official journal of the Society for Neuroscience. 2013 Jun; 33(24):10048-56. doi: 10.1523/jneurosci.0217-13.2013. [PMID: 23761900]
  • Assunta Zanfini, Elena Dreassi, Anna Berardi, Laura Governini, Gianfranco Corbini, Elvira Costantino-Ceccarini, Paolo Balestri, Alice Luddi. Quantification of psychosine in the serum of twitcher mouse by LC-ESI-tandem-MS analysis. Journal of pharmaceutical and biomedical analysis. 2013 Jun; 80(?):44-9. doi: 10.1016/j.jpba.2013.02.039. [PMID: 23523865]
  • Je-Seong Won, Jinsu Kim, Manjeet Kaur Paintlia, Inderjit Singh, Avtar K Singh. Role of endogenous psychosine accumulation in oligodendrocyte differentiation and survival: implication for Krabbe disease. Brain research. 2013 May; 1508(?):44-52. doi: 10.1016/j.brainres.2013.02.024. [PMID: 23438514]
  • 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]
  • Malgorzata J Rybak-Smith, Carla Tripisciano, Ewa Borowiak-Palen, Constanze Lamprecht, Robert B Sim. Effect of functionalization of carbon nanotubes with psychosine on complement activation and protein adsorption. Journal of biomedical nanotechnology. 2011 Dec; 7(6):830-9. doi: 10.1166/jbn.2011.1347. [PMID: 22416583]
  • 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]
  • Ludovico Cantuti Castelvetri, Maria Irene Givogri, Hongling Zhu, Benjamin Smith, Aurora Lopez-Rosas, Xi Qiu, Richard van Breemen, Ernesto Roque Bongarzone. Axonopathy is a compounding factor in the pathogenesis of Krabbe disease. Acta neuropathologica. 2011 Jul; 122(1):35-48. doi: 10.1007/s00401-011-0814-2. [PMID: 21373782]
  • A B White, F Galbiati, M I Givogri, A Lopez Rosas, X Qiu, R van Breemen, E R Bongarzone. Persistence of psychosine in brain lipid rafts is a limiting factor in the therapeutic recovery of a mouse model for Krabbe disease. Journal of neuroscience research. 2011 Mar; 89(3):352-64. doi: 10.1002/jnr.22564. [PMID: 21259322]
  • Maria Blomqvist, Volkmar Gieselmann, Jan-Eric Månsson. Accumulation of lysosulfatide in the brain of arylsulfatase A-deficient mice. Lipids in health and disease. 2011 Feb; 10(?):28. doi: 10.1186/1476-511x-10-28. [PMID: 21299873]
  • Benjamin Smith, Francesca Galbiati, Ludovico Cantuti Castelvetri, Maria I Givogri, Aurora Lopez-Rosas, Ernesto R Bongarzone. Peripheral neuropathy in the Twitcher mouse involves the activation of axonal caspase 3. ASN neuro. 2011; 3(4):. doi: 10.1042/an20110019. [PMID: 21929508]
  • 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]
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