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cyclophellitol and Gaucher Disease

cyclophellitol has been researched along with Gaucher Disease in 5 studies

*Gaucher Disease: An autosomal recessive disorder caused by a deficiency of acid beta-glucosidase (GLUCOSYLCERAMIDASE) leading to intralysosomal accumulation of glycosylceramide mainly in cells of the MONONUCLEAR PHAGOCYTE SYSTEM. The characteristic Gaucher cells, glycosphingolipid-filled HISTIOCYTES, displace normal cells in BONE MARROW and visceral organs causing skeletal deterioration, hepatosplenomegaly, and organ dysfunction. There are several subtypes based on the presence and severity of neurological involvement. [MeSH]

Research

Studies (5)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's1 (20.00)18.2507
2000's0 (0.00)29.6817
2010's4 (80.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Aerts, JM; Aten, J; Bleijlevens, B; Boot, RG; Donker-Koopman, WE; Florea, BI; Hollak, CE; Hooibrink, B; Kallemeijn, WW; Kramer, G; Li, KY; Ottenhoff, R; Overkleeft, HS; Strijland, A; van den Nieuwendijk, AM; van der Marel, GA; Witte, MD1
Aerts, JM; Artola, ME; Kallemeijn, WW; Kuo, CL; Kytidou, K; Overkleeft, HS; van Meel, E; Witte, M1
Aerts, JMFG; Artola, M; Boot, RG; Codée, JDC; Davies, GJ; Kuo, CL; Lelieveld, LT; Overkleeft, HS; Rowland, RJ; van der Marel, GA1
Aerts, JM; Ben Bdira, F; Bleijlevens, B; Boot, R; de Haan, A; Florea, BI; Jiang, J; Kallemeijn, W; Overkleeft, HS; Ubbink, M1
Atsumi, S; Iinuma, H; Nosaka, C; Umezawa, K1

Other Studies

5 other study(ies) available for cyclophellitol and Gaucher Disease

ArticleYear
Ultrasensitive in situ visualization of active glucocerebrosidase molecules.
    Nature chemical biology, 2010, Volume: 6, Issue:12

    Topics: Animals; Boron Compounds; Cells, Cultured; Cyclohexanols; Drug Design; Electrophoresis, Polyacrylamide Gel; Enzyme Inhibitors; Enzyme-Linked Immunosorbent Assay; Fibroblasts; Flow Cytometry; Fluorescent Dyes; Gaucher Disease; Glucosylceramidase; Imino Pyranoses; Mice; Microscopy, Fluorescence; Molecular Chaperones

2010
Activity-Based Probes for Glycosidases: Profiling and Other Applications.
    Methods in enzymology, 2018, Volume: 598

    Topics: Cell Line; Cyclohexanols; Enzyme Assays; Enzyme Inhibitors; Fibroblasts; Fluorescent Dyes; Gaucher Disease; Glucosylceramidase; Glycosphingolipids; Humans; Lysosomes; Microscopy, Confocal; Microscopy, Fluorescence; Molecular Probes; Staining and Labeling

2018
Functionalized Cyclophellitols Are Selective Glucocerebrosidase Inhibitors and Induce a Bona Fide Neuropathic Gaucher Model in Zebrafish.
    Journal of the American Chemical Society, 2019, 03-13, Volume: 141, Issue:10

    Topics: Animals; Cyclohexanols; Disease Models, Animal; Enzyme Inhibitors; Gaucher Disease; Glucosylceramidase; Humans; Zebrafish; Zebrafish Proteins

2019
Hydrophobic Interactions Contribute to Conformational Stabilization of Endoglycoceramidase II by Mechanism-Based Probes.
    Biochemistry, 2016, 08-30, Volume: 55, Issue:34

    Topics: Amino Acid Sequence; Bacterial Proteins; Catalytic Domain; Cyclohexanols; Enzyme Stability; Gaucher Disease; Glucosylceramidase; Glycoside Hydrolases; Humans; Hydrophobic and Hydrophilic Interactions; Models, Molecular; Molecular Probes; Protein Conformation; Recombinant Proteins; Rhodococcus; Structural Homology, Protein

2016
Accumulation of tissue glucosylsphingosine in Gaucher-like mouse induced by the glucosylceramidase inhibitor cyclophellitol.
    Archives of biochemistry and biophysics, 1993, Volume: 304, Issue:1

    Topics: Animals; Brain; Cells, Cultured; Cyclohexanols; Disease Models, Animal; Gaucher Disease; Glucosylceramidase; In Vitro Techniques; Liver; Mice; Psychosine; Spleen

1993