inositol and conduritol epoxide

inositol has been researched along with conduritol epoxide in 97 studies

Research

Studies (97)

TimeframeStudies, this research(%)All Research%
pre-199033 (34.02)18.7374
1990's19 (19.59)18.2507
2000's17 (17.53)29.6817
2010's25 (25.77)24.3611
2020's3 (3.09)2.80

Authors

AuthorsStudies
Hara, A; Radin, NS1
Bernatsky, A; Kanfer, JN; Legler, G; Stephens, MC1
Bernatsky, A; Burachinsky, V; Kanfer, JN; Legler, G; Stephens, MC1
Falchetto, R; Keller, P; Legler, G; Semenza, G; Wacker, H1
Aerts, JM; Barranger, JA; Hong, CM; Ohashi, T; Tager, JM; Tomich, JM; Weiler, S1
Abe, A; Deshmukh, GD; Mahdiyoun, S; Radin, NS; Shayman, JA1
Hermans, MM; Kroos, MA; Oostra, BA; Reuser, AJ; van Beeumen, J1
Maley, F; Reddy, VA1
Butcher, BA; Glew, RH; Gopalan, V; Lee, RE; Richards, TC; Waggoner, AS1
Aerts, JM; Barranger, JA; Donker-Koopman, WE; Koot, M; Schram, AW; Tager, JM1
Bourbouze, R; Donsimoni, R; Lalegerie, P; Legler, G1
McCluer, RH; Newburg, DS; Raghavan, S; Yatziv, S1
Desnick, RJ; Dinur, T; Fabbro, D; Gatt, S; Grabowski, GA; Legler, G; Osiecki-Newman, K1
Datta, SC; Radin, NS1
Basu, A; Das, S; Glew, RH; Lamarco, KL; Legler, G; Martinez, AJ; Nerad, TA; Saha, AK1
Umezurike, GM1
Aerts, JM; Barranger, JA; Magalhaes, JA; Pinto, RA; Sa Miranda, MC; Schram, AW; Tager, JM1
Barfi, G; Kolodny, EH; Livni, N; Newburg, DS; Yatziv, S1
McCluer, RH; Newburg, DS; Raghavan, SS; Shea, TB; Yatziv, S1
Desnick, RJ; Dinur, T; Gatt, S; Grabowski, GA; Legler, G; Osiecki, KM1
Alhadeff, JA; Legler, G; Schray, KJ; White, WJ1
Barranger, JA; Das, PK; Gal, AE; Murray, GJ1
Ge, SG; Yang, SJ; Zeng, YC; Zhang, SZ1
Basu, A; Glew, RH2
Barranger, JA; Das, PK; Murray, GJ1
Dinur, T; Gatt, S; Grabowski, GA; Kruse, JR; Legler, G; Osiecki, KM1
Boyd, SA; Lee, KJ; Radin, NS1
Fiehn, NE; Moe, D1
Herrchen, M; Legler, G2
Radin, NS; Vunnam, RR1
Daniels, LB; Glew, RH; Radin, NS; Vunnam, RR1
Bernatsky, A; Kanfer, JN; Legler, G; Singh, H; Stephens, MC1
Daniels, LB; Diven, WF; Glew, RH; Lee, RE; Radin, NS1
Desnick, RJ; Dinur, T; Gatt, S1
Kanfer, JN; Legler, G; Singh, H; Stephens, MC1
Blair, H; Bull, GM; Gregoriadis, G; Weereratne, H1
Futerman, AH; Hirschberg, K; Rapaport, E; Schwarz, A1
Chiba, S; Honma, M; Ito, H; Iwanami, S; Kimura, A; Matsui, H; Mori, H1
Elias, PM; Holleran, WM; Marsh, NL1
Goto, I; Kobayashi, T; Sasagasako, N; Yamaguchi, Y1
Gatt, S; Grabowski, G; Hirschberg, JG; Kohen, C; Kohen, E; Mangel, W; Prince, J; Santus, R1
Aerts, JM; Brandsma, M; Strijland, A; Tager, JM; van Weely, S1
Chaturvedi, P; Newburg, DS; Prence, EM1
Elias, PM; Feingold, KR; Hanley, K; Holleran, WM; Jiang, Y; Williams, ML1
Barone, R; D'Auria, S; De Napoli, L; Febbraio, F; Nucci, R; Orrù, S; Piccialli, G; Pucci, P; Rossi, M1
Chiba, S; Fukushi, Y; Kimura, A; Matsui, H; Mori, H; Takata, M1
Brown, BE; Elias, PM; Holleran, WM; Marchell, NL; Uchida, Y1
Eghtesad, B; Glew, RH; Hays, WS; Johnston, DE; Wheeler, DE1
Day, AJ; DuPont, MS; Morgan, MR; Rhodes, M; Rhodes, MJ; Ridley, S; Williamson, G1
Futerman, AH; Korkotian, E; Pelled, D; Schwarz, A; Schwarzmann, G; Segal, M1
Futerman, AH; Pelled, D; Shogomori, H1
Falshaw, A; Hart, JB; Tyler, PC1
Chen, FY; Chir, J; Li, YK1
Aouali, N; Belhoussine, R; Levade, T; Manfait, M; Morjani, H; Veldman, RJ1
De Gori, R; Driguez, H; Fincher, GB; Hrmova, M; Smith, BJ; Varghese, JN1
Bodennec, J; Futerman, AH; Pelled, D; Riebeling, C; Trajkovic, S1
Chiba, S; Fukuda, K; Kimura, A; Mori, H; Okuyama, M; Ozaki, H; Yoneyama, M1
Andrews, L; Chan, KW; Copeland, D; Fung, J; Karey, K; Simons, B; Waire, J1
Brady, RO; Kaneski, CR; Kolter, T; Sandhoff, K; Schueler, UH; Zirzow, GC1
Casas, J; Delgado, A; Egido-Gabás, M; Llebaria, A; Serrano, P1
Boldin-Adamsky, S; Futerman, AH; Kelly, JW; Premkumar, L; Sawkar, AR; Silman, I; Sussman, JL; Toker, L1
Delgado, A; Llebaria, A; Serrano, P1
Hayashi, Y; Ito, M; Kakuta, Y; Narimatsu, H; Okino, N; Shikanai, T; Tani, M1
Grabowski, GA; Huelsken, J; Quinn, B; Reboulet, R; Witte, D; Xu, YH1
Anelli, V; Grabowski, GA; Hannun, YA; Jenkins, RW; Kitatani, K; Obeid, LM; Sheldon, K; Sun, Y1
Langston, JW; Manning-Boğ, AB; Schüle, B1
Boivin, MN; Campeau, PM; Galipeau, J; Grabowski, GA; Rafei, M; Sun, Y1
Arosa, FA; Balreira, A; Cavallari, M; Sá Miranda, MC1
Berger, J; Berger, MG; Boiret-Dupré, N; Boisgard, S; Caillaud, C; Chomienne, C; Larghero, J; Lecourt, S; Marolleau, JP; Rapatel, C; Vanneaux, V1
Grabowski, GA; Quinn, B; Ran, H; Sun, Y; Witte, D; Xu, YH1
Goldin, E; Gruschus, JM; Lee, JC; Moaven, N; Sidransky, E; Velayati, A; Westbroek, W; Yap, TL1
Belmatoug, N; Caillaud, C; Chomienne, C; Cras, A; Freida, D; Heraoui, D; Herbi, L; Larghero, J; Lecourt, S; Marolleau, JP; Vanneaux, V1
Imokawa, G; Sawada, E; Yoshida, N1
Chau, KY; Cleeter, MW; Duchen, M; Gluck, C; Hardy, J; Hughes, DA; Mark Cooper, J; Mehta, A; Schapira, AH; Wood, NW1
Dermentzaki, G; Dimitriou, E; Michelakakis, H; Stefanis, L; Xilouri, M1
Platt, FM; Rahim, AA; Ridley, CM; Shanahan, J; Shen, A; Thillaiappan, NB; Thur, KE; Uhl, K; van der Spoel, AC; Waddington, SN; Walden, CM1
Ceci, R; de Francesco, PN; Delpino, MV; Di Genaro, S; Fossati, CA; Mucci, JM; Rozenfeld, PA; Suqueli García, F1
Akbarian, S; Chou, VP; Ginns, EI; Guo, Y; Karlgren, J; Ko, N; LaMarca, ML; Lim, A; Mak, SK; Manning-Boğ, AB; Samuelsson, S; Vazquez-DeRose, J1
Grabowski, GA; Li, RH; Liou, B; Quinn, B; Setchell, KD; Sun, Y; Witte, D; Xu, K; Xu, YH; Xue, L; Zhang, W1
Beagan, J; Brown, E; Cao, H; Graham, AR; Hallett, PJ; Hayes, MA; Isacson, O; Izen, SC; McLean, JR; Park, E; Perez-Torres, E; Rocha, EM; Smith, GA1
Alvarez-Fischer, D; Andreas, H; Hirsch, EC; Höglinger, GU; Höllerhage, M; Lu, L; Noelker, C; Oertel, WH; Roscher, R; Sturn, A; Vulinovic, F1
Allaire, A; Bangari, DS; Budman, E; Cheng, SH; Cromwell, MA; Grabowski, GA; Leonard, JP; Marshall, J; Nietupski, JB; Park, H; Sun, Y; Wang, B1
Brayer, GD; Caner, S; Chen, HM; Jiang, J; Nguyen, NT; Overkleeft, H; Withers, SG; Zhang, X1
Hayashi, Y; Ito, M1
Eilam, R; Futerman, AH; Kenwood, BM; Klein, AD; Massaro, G; Merrill, AH; Meshcheriakova, A; Rahim, AA; Vardi, A; Vitner, EB; Yaacobi, C; Zigdon, H1
Ben-Dor, S; Cox, TM; Duan, J; Ferreira, NS; Futerman, AH; Hardy, J; Klein, AD; Merrill, AH1
Bondar, C; Crivaro, A; Delpino, MV; Ferreyra-Compagnucci, M; Mucci, JM; Ormazabal, M; Rozenfeld, PA1
Burke, DG; de la Fuente, C; Eaton, S; Heales, SJR1
Antoniadou, I; Arianoglou, G; Emmanouilidou, E; Karachaliou, A; Nikolopoulou, G; Papadopoulos, VE; Sardi, SP; Stefanis, L; Vekrellis, K1
Blandini, F; Cerri, S; Ghezzi, C; Giuliano, C; Mus, L; Siani, F1
Aerts, JMFG; Artola, M; Futerman, AH; Kallemeijn, WW; Kuo, CL; Lelieveld, LT; Meijer, AH; Mirzaian, M; Overkleeft, HS; Spaink, HP; Vardi, A; Zoutendijk, I1
Chan, MY; Svenningsson, P; Zurbruegg, M1
Castro, H; Macedo, MF; Ribeiro, H; Rocha, MI1
Aivazidis, S; Burbidge, KJ; Fredriksen, K; Gelyana, E; Mazzulli, JR; Pitcairn, C; Sharma, K; Zunke, F1
Alcantud, JL; Arrebola, F; Duran, R; Hardy, J; Lewis, PA; Manzoni, C; Ruz, C; Vives, F1

Reviews

1 review(s) available for inositol and conduritol epoxide

ArticleYear
Klotho-Related Protein KLrP: Structure and Functions.
    Vitamins and hormones, 2016, Volume: 101

    Topics: Animals; Brain; Crystallography, X-Ray; Cytosol; Fibroblasts; Gaucher Disease; Gene Knockdown Techniques; Glucosylceramidase; Glucosylceramides; Glucuronidase; Humans; Hydrogen-Ion Concentration; Inositol; Klotho Proteins; Models, Molecular; Molecular Structure; Mutation; Rats; Zebrafish

2016

Other Studies

96 other study(ies) available for inositol and conduritol epoxide

ArticleYear
Destruction and resynthesis of mouse beta-glucosidases.
    Biochimica et biophysica acta, 1979, Feb-01, Volume: 582, Issue:3

    Topics: Animals; beta-Glucosidase; Brain; Glucosidases; Glucosylceramidase; Hematocrit; Hydrogen-Ion Concentration; Inositol; Kidney; Liver; Organ Size; Phenylhydrazines; Spleen

1979
Studies on the possible identity of particulate beta-glucosidase and beta-xylosidase of mouse liver.
    Biochimica et biophysica acta, 1979, Nov-09, Volume: 571, Issue:1

    Topics: Animals; beta-Glucosidase; Gluconates; Glucosidases; Glycoside Hydrolases; Hydrogen-Ion Concentration; Inositol; Kinetics; Lactones; Liver; Mice; Taurocholic Acid; Xylosidases

1979
The Gaucher mouse: differential action of conduritol B epoxide and reversibility of its effects.
    Journal of neurochemistry, 1978, Volume: 30, Issue:5

    Topics: Acetylglucosaminidase; Animals; beta-Glucosidase; Brain; Cerebrosides; Gaucher Disease; Humans; Inositol; Liver; Male; Mice; Spleen

1978
Location of the two catalytic sites in intestinal lactase-phlorizin hydrolase. Comparison with sucrase-isomaltase and with other glycosidases, the membrane anchor of lactase-phlorizin hydrolase.
    The Journal of biological chemistry, 1992, Sep-15, Volume: 267, Issue:26

    Topics: 1-Deoxynojirimycin; Amino Acid Sequence; Animals; beta-Galactosidase; Binding Sites; Catalysis; DNA; Glucosamine; Glycoside Hydrolases; Inositol; Intestines; Lactase; Lactase-Phlorizin Hydrolase; Microvilli; Molecular Sequence Data; Oligo-1,6-Glucosidase; Rabbits; Sequence Homology, Nucleic Acid; Sucrase

1992
Characterization of human glucocerebrosidase from different mutant alleles.
    The Journal of biological chemistry, 1991, Feb-25, Volume: 266, Issue:6

    Topics: Alleles; Blotting, Northern; Blotting, Western; Chromatography, Affinity; DNA; Glucosylceramidase; Glycoproteins; Hot Temperature; Humans; Inositol; Mutagenesis, Site-Directed; Mutation; Phosphatidylserines; RNA; Saposins; Transfection

1991
Decreased formation of inositol trisphosphate in Madin-Darby canine kidney cells under conditions of beta-glucosidase inhibition.
    Archives of biochemistry and biophysics, 1992, Feb-01, Volume: 292, Issue:2

    Topics: Animals; beta-Glucosidase; Bradykinin; Cell Line; Dogs; Galactose; Glucosylceramides; Guanosine 5'-O-(3-Thiotriphosphate); Inositol; Inositol 1,4,5-Trisphosphate; Kidney; Kinetics; Palmitic Acid; Palmitic Acids; Radioisotope Dilution Technique; Sphingolipids; Tritium

1992
Human lysosomal alpha-glucosidase. Characterization of the catalytic site.
    The Journal of biological chemistry, 1991, Jul-25, Volume: 266, Issue:21

    Topics: alpha-Glucosidases; Amino Acid Sequence; Base Sequence; Binding Sites; Chromatography, High Pressure Liquid; DNA Mutational Analysis; Glycoside Hydrolase Inhibitors; Humans; Inositol; Lysosomes; Molecular Sequence Data; Oligonucleotides; Peptide Fragments; Protein Processing, Post-Translational; Structure-Activity Relationship

1991
Identification of an active-site residue in yeast invertase by affinity labeling and site-directed mutagenesis.
    The Journal of biological chemistry, 1990, Jul-05, Volume: 265, Issue:19

    Topics: Acetylglucosaminidase; Affinity Labels; Amino Acid Sequence; Aspartic Acid; Base Sequence; beta-Fructofuranosidase; Binding Sites; Catalysis; Chromatography, High Pressure Liquid; Glycoside Hydrolases; Glycosylation; Inositol; Kinetics; Mannosyl-Glycoprotein Endo-beta-N-Acetylglucosaminidase; Molecular Sequence Data; Mutation; Peptide Fragments; Saccharomyces cerevisiae; Trypsin

1990
Use of 4-heptylumbelliferyl-beta-D-glucoside to identify Gaucher's disease heterozygotes.
    Clinica chimica acta; international journal of clinical chemistry, 1989, Oct-16, Volume: 184, Issue:3

    Topics: beta-Glucosidase; Brain; Gaucher Disease; Genetic Carrier Screening; Glucosylceramidase; Humans; Hydrogen-Ion Concentration; Hydrolysis; Immunoenzyme Techniques; Inositol; Leukocytes; Liver; Placenta; Taurocholic Acid; Umbelliferones

1989
Deficient activity of glucocerebrosidase in urine from patients with type 1 Gaucher disease.
    Clinica chimica acta; international journal of clinical chemistry, 1986, Jul-30, Volume: 158, Issue:2

    Topics: Adolescent; Adult; beta-N-Acetylhexosaminidases; Child; Female; Gaucher Disease; Glucosidases; Glucosides; Glucosylceramidase; Hexosaminidases; Humans; Hymecromone; Inositol; Male; Middle Aged; Substrate Specificity

1986
Study of beta-glucosidase from Helix pomatia by active site-directed inhibitors.
    Enzyme, 1988, Volume: 39, Issue:2

    Topics: 1-Deoxynojirimycin; Animals; beta-Glucosidase; Glucosamine; Glucosidases; Helix, Snails; Hydrogen-Ion Concentration; Inositol; Kinetics; Substrate Specificity

1988
beta-Glucosidase inhibition in murine peritoneal macrophages by conduritol-B-epoxide: an in vitro model of the Gaucher cell.
    Biochimica et biophysica acta, 1986, Jun-11, Volume: 877, Issue:1

    Topics: Animals; beta-Glucosidase; Cells, Cultured; Dose-Response Relationship, Drug; Gaucher Disease; Glucosidases; Glucosylceramides; Inositol; Macrophages; Mice; Models, Biological; Peritoneum; Time Factors

1986
Human acid beta-glucosidase. Use of conduritol B epoxide derivatives to investigate the catalytically active normal and Gaucher disease enzymes.
    The Journal of biological chemistry, 1986, Jun-25, Volume: 261, Issue:18

    Topics: beta-Glucosidase; Gaucher Disease; Glucosidases; Humans; Immunoglobulin G; Inositol; Kinetics; Mathematics; Placenta

1986
Glucosylceramide and the level of the glucosidase-stimulating proteins.
    Lipids, 1986, Volume: 21, Issue:11

    Topics: Animals; beta-Glucosidase; Body Weight; Brain; Cell Membrane; Cerebrosides; Cytosol; Glucosidases; Glucosylceramides; Glycoproteins; Inositol; Kinetics; Liver; Mice; Mice, Inbred Strains; Organ Size; Proteins; Saposins

1986
Partial purification and characterization of Naegleria fowleri beta-glucosidase.
    The Journal of protozoology, 1987, Volume: 34, Issue:1

    Topics: Amoeba; Animals; beta-Glucosidase; Chromatography, Gel; Disaccharides; Glucosidases; Hot Temperature; Hydrogen-Ion Concentration; Inositol; Isoelectric Focusing; Kinetics; Microscopy, Electron; Substrate Specificity

1987
The mechanism of action of beta-glucosidase from Botryodiplodia theobromae Pat.
    The Biochemical journal, 1987, Jan-15, Volume: 241, Issue:2

    Topics: beta-Glucosidase; Dioxanes; Glucosidases; Glycerol; Inositol; Kinetics; Mitosporic Fungi; Sodium Chloride

1987
Heterogeneity in human acid beta-glucosidase revealed by cellulose-acetate electrophoresis.
    Biochimica et biophysica acta, 1988, May-12, Volume: 965, Issue:2-3

    Topics: beta-Glucosidase; Electrophoresis; Electrophoresis, Cellulose Acetate; Female; Fibroblasts; Gaucher Disease; Glucosidases; Glucosylceramidase; Glycoproteins; Humans; Inositol; Isoelectric Point; Kidney; Leukocytes; Liver; Phosphatidylserines; Placenta; Pregnancy; Saposins; Solvents; Sphingolipid Activator Proteins; Spleen; Taurocholic Acid

1988
Gaucher-like changes in human blood-derived macrophages induced by beta-glucocerebrosidase inhibition.
    The Journal of laboratory and clinical medicine, 1988, Volume: 111, Issue:4

    Topics: Blood Cells; Gaucher Disease; Glucosidases; Glucosylceramidase; Humans; Inositol; Macrophages; Microscopy, Electron

1988
Macrophages exposed in vitro to conduritol B epoxide resemble Gaucher cells.
    Experimental and molecular pathology, 1988, Volume: 48, Issue:3

    Topics: Animals; Gaucher Disease; Glucosidases; Glucosylceramidase; Glucosylceramides; Inositol; Lysosomes; Macrophages; Mice; Mice, Inbred C57BL

1988
Human acid beta-glucosidase: isolation and amino acid sequence of a peptide containing the catalytic site.
    Proceedings of the National Academy of Sciences of the United States of America, 1986, Volume: 83, Issue:6

    Topics: Amino Acid Sequence; Base Sequence; Binding Sites; DNA; Gaucher Disease; Glucosidases; Glucosylceramidase; Humans; Inositol; Lysosomes; Peptides; Sequence Homology, Nucleic Acid

1986
Active-site-directed inactivation of human liver alpha-L-fucosidase by conduritol C trans-epoxide.
    Biochimica et biophysica acta, 1986, Sep-26, Volume: 873, Issue:2

    Topics: alpha-L-Fucosidase; Binding Sites; Humans; Hydrogen-Ion Concentration; Inositol; Kinetics; Liver; Stereoisomerism

1986
Glucocerebrosidase deficiency and lysosomal storage of glucocerebroside induced in cultured macrophages.
    Experimental cell research, 1987, Volume: 168, Issue:2

    Topics: Animals; Carbon Radioisotopes; Cells, Cultured; Cerebrosides; Glucosidases; Glucosylceramidase; Glucosylceramides; Inositol; Kinetics; Liposomes; Lysosomes; Macrophages; Rats

1987
Inactivation of alpha-glucosidase by the active-site-directed inhibitor, conduritol B epoxide.
    Biochimica et biophysica acta, 1985, Apr-29, Volume: 828, Issue:3

    Topics: Binding Sites; Glucosidases; Glycoside Hydrolase Inhibitors; Hydrogen-Ion Concentration; Inositol; Kinetics; Maltose

1985
Characterization of the activation of rat liver beta-glucosidase by sialosylgangliotetraosylceramide.
    The Journal of biological chemistry, 1985, Oct-25, Volume: 260, Issue:24

    Topics: Animals; beta-Glucosidase; Chemical Phenomena; Chemistry, Physical; Dose-Response Relationship, Drug; Enzyme Activation; G(M1) Ganglioside; Gangliosides; Gaucher Disease; Glucosidases; Glucosides; Hot Temperature; Humans; Hymecromone; Inositol; Kinetics; Liver; Lysosomes; Male; Micelles; Molecular Weight; Rats; Rats, Inbred Strains; Temperature

1985
Studies on the turnover of glucocerebrosidase in cultured rat peritoneal macrophages and normal human fibroblasts.
    European journal of biochemistry, 1986, Jan-15, Volume: 154, Issue:2

    Topics: Animals; Cells, Cultured; Chemical Precipitation; Fibroblasts; Glucosidases; Glucosylceramidase; Humans; Immunochemistry; Inositol; Kinetics; Macrophages; Peritoneal Cavity; Rats

1986
Gaucher disease types 1, 2, and 3: differential mutations of the acid beta-glucosidase active site identified with conduritol B epoxide derivatives and sphingosine.
    American journal of human genetics, 1985, Volume: 37, Issue:3

    Topics: Adolescent; Adult; Binding Sites; Cells, Cultured; Child; Child, Preschool; Female; Fibroblasts; Gaucher Disease; Glucosidases; Glucosylceramidase; Humans; Inositol; Jews; Male; Middle Aged; Mutation; Phenotype; Sphingosine

1985
Improved synthesis of conduritol B epoxide.
    Carbohydrate research, 1985, Nov-15, Volume: 144, Issue:1

    Topics: Chemical Phenomena; Chemistry; Chromatography, Gas; Chromatography, Thin Layer; Indicators and Reagents; Inositol

1985
Effect of seven inhibitors on invertases in homogenates of human dental plaque.
    Scandinavian journal of dental research, 1983, Volume: 91, Issue:3

    Topics: 1-Deoxynojirimycin; Acarbose; beta-Fructofuranosidase; Dental Plaque; Glucosamine; Glucosidases; Glycoside Hydrolase Inhibitors; Glycoside Hydrolases; Humans; Inositol; Peptides; Piperidones; Trisaccharides

1983
Active site-directed inhibition of galactosidases by conduritol C epoxides (1,2-anhydro-epi- and neo-inositol).
    FEBS letters, 1981, Nov-30, Volume: 135, Issue:1

    Topics: alpha-Galactosidase; Animals; beta-Galactosidase; beta-Glucosidase; Cattle; Escherichia coli; Galactosidases; Helix, Snails; Inositol; Kinetics; Plants; Spleen; Structure-Activity Relationship

1981
Identification of an essential carboxylate group at the active site of lacZ beta-galactosidase from Escherichia coli.
    European journal of biochemistry, 1984, Feb-01, Volume: 138, Issue:3

    Topics: beta-Galactosidase; Binding Sites; Borohydrides; Carboxylic Acids; Catalysis; Chemical Phenomena; Chemistry; Escherichia coli; Galactosidases; Inositol

1984
Inhibitors of cerebroside metabolism.
    Methods in enzymology, 1981, Volume: 72

    Topics: Animals; Cerebrosides; Galactosylceramidase; Glucosylceramidase; Glucosyltransferases; Indicators and Reagents; Inositol; Morpholines; Propiophenones; Propylene Glycols; Sphingosine

1981
Characterization of the phospholipid requirement of a rat liver beta-glucosidase.
    The Biochemical journal, 1984, Dec-01, Volume: 224, Issue:2

    Topics: Animals; Enzyme Activation; Glucosidases; Glucosylceramidase; Hydrogen-Ion Concentration; Inositol; Kinetics; Liver; Male; Phosphatidylserines; Phospholipids; Proteins; Rats; Rats, Inbred Strains

1984
A revised fluorometric assay for Gaucher's disease using conduritol-beta-epoxide with liver as the source of Beta-glucosidase.
    Clinica chimica acta; international journal of clinical chemistry, 1980, Sep-25, Volume: 106, Issue:2

    Topics: beta-Glucosidase; Clinical Enzyme Tests; Fluorometry; Gaucher Disease; Glucosidases; Glucosylceramidase; Humans; Inositol; Liver

1980
Distribution of conduritol B epoxide in the animal model for Gaucher's disease (Gaucher mouse).
    Biochimica et biophysica acta, 1981, Jan-07, Volume: 672, Issue:1

    Topics: Animals; beta-Glucosidase; Blood-Brain Barrier; Disease Models, Animal; Gaucher Disease; Glucosidases; Humans; Inositol; Mice; Tissue Distribution

1981
An improved fluorometric leukocyte beta-glucosidase assay for Gaucher's disease.
    Clinica chimica acta; international journal of clinical chemistry, 1981, Volume: 115, Issue:3

    Topics: Adolescent; beta-Glucosidase; Clinical Enzyme Tests; Female; Fibroblasts; Fluorometry; Gaucher Disease; Genetic Carrier Screening; Glucosidases; Glucosylceramidase; Homozygote; Humans; Inositol; Leukocytes; Male; Taurocholic Acid

1981
Studies on human acid beta-glucosidase and the nature of the molecular defect in type 1 Ashkenazi Gaucher disease.
    Progress in clinical and biological research, 1982, Volume: 95

    Topics: beta-Glucosidase; Detergents; Europe; Gaucher Disease; Glucosidases; Glucosides; Humans; Hydrolysis; Inositol; Jews; Psychosine

1982
The Gaucher mouse.
    Progress in clinical and biological research, 1982, Volume: 95

    Topics: Acid Phosphatase; Animals; beta-Glucosidase; Brain; Cerebrosides; Disease Models, Animal; Galactosylceramides; Gangliosides; Gaucher Disease; Humans; Inositol; Mice; Myelin Sheath; Spleen

1982
Liposomes in Gaucher type I disease: use in enzyme therapy and the creation of an animal model.
    Progress in clinical and biological research, 1982, Volume: 95

    Topics: Adult; Animals; beta-Glucosidase; Disease Models, Animal; Female; Gaucher Disease; Glucosidases; Glucosylceramidase; Humans; Inositol; Liposomes; Mice; Mice, Inbred BALB C

1982
A regulatory role for sphingolipids in neuronal growth. Inhibition of sphingolipid synthesis and degradation have opposite effects on axonal branching.
    The Journal of biological chemistry, 1995, May-05, Volume: 270, Issue:18

    Topics: Animals; Axons; Cells, Cultured; Dendrites; Fumonisins; Hippocampus; In Vitro Techniques; Inositol; Morpholines; Mycotoxins; Neurons; Rats; Rats, Wistar; Sphingolipids

1995
Chemical modification and amino acid sequence of active site in sugar beet alpha-glucosidase.
    Bioscience, biotechnology, and biochemistry, 1995, Volume: 59, Issue:3

    Topics: alpha-Glucosidases; Amino Acid Sequence; beta-Glucosidase; Chromatography, High Pressure Liquid; Electrophoresis; Glycoside Hydrolase Inhibitors; Inositol; Kinetics; Molecular Sequence Data; Plants; Protein Conformation; Ultracentrifugation

1995
Glucosylceramides stimulate murine epidermal hyperproliferation.
    The Journal of clinical investigation, 1995, Volume: 95, Issue:6

    Topics: Animals; Cell Division; Epidermal Cells; Glucosylceramidase; Glucosylceramides; Inflammation; Inositol; Male; Mice; Sphingolipids

1995
The synthetic pathway for glucosylsphingosine in cultured fibroblasts.
    Journal of biochemistry, 1994, Volume: 116, Issue:3

    Topics: Acylation; beta-Glucosidase; Cells, Cultured; Cerebrosides; Fibroblasts; Glucosylceramides; Glucosyltransferases; Glycosylation; Humans; Inositol; Morpholines; Psychosine; Sphingosine

1994
An in situ study of beta-glucosidase activity in normal and Gaucher fibroblasts with fluorogenic probes.
    Cell biochemistry and function, 1993, Volume: 11, Issue:3

    Topics: beta-Galactosidase; beta-Glucosidase; Cells, Cultured; Fibroblasts; Fluorescent Dyes; Gaucher Disease; Glycosides; Homozygote; Humans; Inositol; Lysosomes; Microscopy, Fluorescence; Molecular Probes; Spectrometry, Fluorescence

1993
Demonstration of the existence of a second, non-lysosomal glucocerebrosidase that is not deficient in Gaucher disease.
    Biochimica et biophysica acta, 1993, Mar-24, Volume: 1181, Issue:1

    Topics: beta-Glucosidase; Cells, Cultured; Gaucher Disease; Glucosylceramidase; Humans; Hydrogen-Ion Concentration; Inositol; Isoenzymes; Lysosomes

1993
In vitro accumulation of glucocerebroside in neuroblastoma cells: a model for study of Gaucher disease pathobiology.
    Journal of neuroscience research, 1996, Feb-01, Volume: 43, Issue:3

    Topics: alpha-Mannosidase; beta-Galactosidase; beta-Glucosidase; Cell Line; Gaucher Disease; Glucosylceramidase; Glucosylceramides; Glycolipids; Humans; Inositol; Kinetics; Lysosomes; Mannosidases; Microscopy, Electron; Models, Biological; Neuroblastoma; Tumor Cells, Cultured

1996
Glucosylceramide metabolism is regulated during normal and hormonally stimulated epidermal barrier development in the rat.
    Journal of lipid research, 1997, Volume: 38, Issue:3

    Topics: Animals; Dexamethasone; Diethylstilbestrol; Embryonic and Fetal Development; Enzyme Inhibitors; Epidermis; Gestational Age; Glucosylceramidase; Glucosylceramides; Glucosyltransferases; Hormones; Inositol; Microscopy, Electron; Organ Culture Techniques; Permeability; Rats; Rats, Sprague-Dawley; RNA, Messenger; Triiodothyronine

1997
Identification of the active site nucleophile in the thermostable beta-glycosidase from the archaeon Sulfolobus solfataricus expressed in Escherichia coli.
    Biochemistry, 1997, Mar-18, Volume: 36, Issue:11

    Topics: Amino Acid Sequence; beta-Glucosidase; Binding Sites; Cloning, Molecular; Enzyme Inhibitors; Escherichia coli; Glucosidases; Glycoside Hydrolases; Inositol; Kinetics; Mass Spectrometry; Models, Molecular; Molecular Sequence Data; Protein Conformation; Recombinant Proteins; Sequence Homology, Amino Acid; Sulfolobus

1997
A catalytic amino acid and primary structure of active site in Aspergillus niger alpha-glucosidase.
    Bioscience, biotechnology, and biochemistry, 1997, Volume: 61, Issue:7

    Topics: alpha-Glucosidases; Amino Acid Sequence; Aspartic Acid; Aspergillus niger; Binding Sites; Enzyme Inhibitors; Inositol; Kinetics; Molecular Sequence Data; Sequence Analysis; Sequence Homology, Amino Acid; Structure-Activity Relationship

1997
Glucosylceramides stimulate mitogenesis in aged murine epidermis.
    The Journal of investigative dermatology, 1998, Volume: 110, Issue:4

    Topics: Administration, Topical; Aging; Animals; Animals, Newborn; DNA; Enzyme Inhibitors; Epidermal Cells; Epidermis; Glucosylceramidase; Glucosylceramides; Hyperplasia; Inositol; Male; Mice; Mice, Hairless; Mitogens

1998
Expression of cytosolic beta-glucosidase in guinea pig liver cells.
    Hepatology (Baltimore, Md.), 1998, Volume: 28, Issue:1

    Topics: Animals; beta-Glucosidase; Carbon Tetrachloride; Cells, Cultured; Cytosol; Enzyme Inhibitors; Extracellular Space; Female; Glucosides; Guinea Pigs; Hydrolysis; Inositol; Liver; Male

1998
Deglycosylation of flavonoid and isoflavonoid glycosides by human small intestine and liver beta-glucosidase activity.
    FEBS letters, 1998, Sep-25, Volume: 436, Issue:1

    Topics: beta-Glucosidase; Cell Extracts; Cell-Free System; Chamomile; Cytosol; Flavanones; Flavonoids; Genistein; Gluconates; Glycosides; Glycosylation; Humans; Inositol; Intestine, Small; Isoflavones; Lactones; Liver; Oils, Volatile; Plants, Medicinal; Quercetin; Rutin; Taurocholic Acid

1998
Elevation of intracellular glucosylceramide levels results in an increase in endoplasmic reticulum density and in functional calcium stores in cultured neurons.
    The Journal of biological chemistry, 1999, Jul-30, Volume: 274, Issue:31

    Topics: Animals; Calcium; Cells, Cultured; Embryo, Mammalian; Endoplasmic Reticulum; Gangliosides; Glucosylceramidase; Glucosylceramides; Hippocampus; Inositol; Kinetics; Lysosomes; Neurons; Rats; Rats, Wistar; Time Factors

1999
The increased sensitivity of neurons with elevated glucocerebroside to neurotoxic agents can be reversed by imiglucerase.
    Journal of inherited metabolic disease, 2000, Volume: 23, Issue:2

    Topics: Animals; Antimetabolites; Carboxylic Acids; Cell Death; Cells, Cultured; Enzyme Inhibitors; Fumonisins; Gaucher Disease; Glucosylceramidase; Glucosylceramides; Hippocampus; Inositol; Neurons; Neurotoxins; Rats; Rats, Wistar; Recombinant Proteins

2000
New syntheses of 1D- and 1L-1,2-anhydro-myo-inositol and assessment of their glycosidase inhibitory activities.
    Carbohydrate research, 2000, Nov-03, Volume: 329, Issue:2

    Topics: Aspergillus oryzae; Bacterial Proteins; beta-Galactosidase; beta-Glucosidase; Carbohydrate Conformation; Enzyme Inhibitors; Fungal Proteins; Geobacillus stearothermophilus; Glycoside Hydrolase Inhibitors; Glycoside Hydrolases; Indolizines; Inositol; Magnetic Resonance Spectroscopy; Plant Proteins; Stereoisomerism; Structure-Activity Relationship; Substrate Specificity

2000
Catalytic mechanism of a family 3 beta-glucosidase and mutagenesis study on residue Asp-247.
    The Biochemical journal, 2001, May-01, Volume: 355, Issue:Pt 3

    Topics: Aspartic Acid; beta-Glucosidase; Catalysis; Enzyme Inhibitors; Flavobacterium; Inositol; Kinetics; Magnetic Resonance Spectroscopy; Mutagenesis, Site-Directed; Substrate Specificity

2001
Elevation of glucosylceramide in multidrug-resistant cancer cells and accumulation in cytoplasmic droplets.
    International journal of cancer, 2001, Oct-15, Volume: 94, Issue:2

    Topics: Cell Nucleus; Cytoplasm; Daunorubicin; Drug Resistance, Multiple; Drug Resistance, Neoplasm; Glucosylceramides; Humans; Inositol; Microscopy, Confocal; Neoplasms; Tumor Cells, Cultured

2001
Catalytic mechanisms and reaction intermediates along the hydrolytic pathway of a plant beta-D-glucan glucohydrolase.
    Structure (London, England : 1993), 2001, Volume: 9, Issue:11

    Topics: Catalytic Domain; Crystallography, X-Ray; Cyclohexanols; Fluorodeoxyglucose F18; Glucose; Glucosidases; Hordeum; Hydrolysis; Inositol; Models, Chemical; Models, Molecular; Peptide Mapping; Quantum Theory

2001
Phosphatidylcholine synthesis is elevated in neuronal models of Gaucher disease due to direct activation of CTP:phosphocholine cytidylyltransferase by glucosylceramide.
    FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2002, Volume: 16, Issue:13

    Topics: Animals; Axons; Brain; Carbon Radioisotopes; Cell Division; Choline; Choline-Phosphate Cytidylyltransferase; Enzyme Activation; Enzyme Inhibitors; Galactosylceramides; Gaucher Disease; Genotype; Glucosylceramidase; Glucosylceramides; Inositol; Lipid Metabolism; Mice; Mutation; Neurons; Phosphatidylcholines; Rats

2002
Identification of essential ionizable groups and evaluation of subsite affinities in the active site of beta-D-glucosidase F1 from a Streptomyces sp.
    Bioscience, biotechnology, and biochemistry, 2002, Volume: 66, Issue:10

    Topics: Algorithms; Amino Acid Sequence; beta-Glucosidase; Binding Sites; Catalysis; Hydrogen-Ion Concentration; Inositol; Kinetics; Molecular Sequence Data; Oligosaccharides; Streptomyces; Substrate Specificity; Temperature

2002
Measurement of lysosomal glucocerebrosidase activity in mouse liver using a fluorescence-activated cell sorter assay.
    Analytical biochemistry, 2004, Nov-15, Volume: 334, Issue:2

    Topics: Animals; Cells, Cultured; Dose-Response Relationship, Drug; Female; Flow Cytometry; Glucosylceramidase; Inositol; Liver; Lysosomes; Mice; Mice, Inbred BALB C; Reproducibility of Results

2004
Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease.
    Journal of inherited metabolic disease, 2004, Volume: 27, Issue:5

    Topics: Animals; Cell Culture Techniques; Cell Line; Chromatography, High Pressure Liquid; Dose-Response Relationship, Drug; Enzyme Inhibitors; Erythrocyte Membrane; Gaucher Disease; Glucosylceramidase; Glucosylceramides; Heterozygote; Inositol; Lipid Metabolism; Macrophages; Mice; Microscopy, Electron

2004
New aminocyclitols as modulators of glucosylceramide metabolism.
    Organic & biomolecular chemistry, 2005, Apr-07, Volume: 3, Issue:7

    Topics: Enzyme Inhibitors; Glucosidases; Glucosylceramides; Hexosamines; Inositol; Kinetics; Magnetic Resonance Spectroscopy; Models, Molecular; Optical Rotation; Spectrometry, Mass, Electrospray Ionization; Structure-Activity Relationship

2005
X-ray structure of human acid-beta-glucosidase covalently bound to conduritol-B-epoxide. Implications for Gaucher disease.
    The Journal of biological chemistry, 2005, Jun-24, Volume: 280, Issue:25

    Topics: beta-Glucosidase; Binding Sites; Catalysis; Crystallography, X-Ray; Gaucher Disease; Humans; Inositol; Models, Molecular

2005
Regio- and stereoselective synthesis of aminoinositols and 1,2-diaminoinositols from conduritol B epoxide.
    The Journal of organic chemistry, 2005, Sep-30, Volume: 70, Issue:20

    Topics: Amines; Epoxy Compounds; Indicators and Reagents; Inositol; Magnetic Resonance Spectroscopy; Models, Molecular; Stereoisomerism

2005
Klotho-related protein is a novel cytosolic neutral beta-glycosylceramidase.
    The Journal of biological chemistry, 2007, Oct-19, Volume: 282, Issue:42

    Topics: Animals; Cell Line; Cricetinae; Cricetulus; Crystallography, X-Ray; Cytosol; Drug Resistance; Fibroblasts; Gene Expression; Glucose; Glucosylceramidase; Glucosylceramides; Glucuronidase; Humans; Inositol; Klotho Proteins; Lysosomes; Mice; Protein Structure, Secondary; Protein Structure, Tertiary; Rats; Recombinant Proteins; RNA, Small Interfering; Zebrafish; Zebrafish Proteins

2007
Dependence of reversibility and progression of mouse neuronopathic Gaucher disease on acid beta-glucosidase residual activity levels.
    Molecular genetics and metabolism, 2008, Volume: 94, Issue:2

    Topics: Animals; Central Nervous System; Cytokines; Disease Models, Animal; Disease Progression; Female; Gaucher Disease; Glucosylceramidase; Glucosylceramides; Glycosphingolipids; Inositol; Keratinocytes; Male; Mice; Mice, Transgenic; Phenotype

2008
Acid beta-glucosidase 1 counteracts p38delta-dependent induction of interleukin-6: possible role for ceramide as an anti-inflammatory lipid.
    The Journal of biological chemistry, 2009, May-08, Volume: 284, Issue:19

    Topics: Blotting, Western; Breast Neoplasms; Carcinogens; Cell Line, Tumor; Ceramides; Enzyme-Linked Immunosorbent Assay; Female; Gene Silencing; Glucosylceramidase; Humans; Inositol; Interleukin-6; Mitogen-Activated Protein Kinase 13; Phosphorylation; Protein Kinase C; Reverse Transcriptase Polymerase Chain Reaction; RNA, Messenger; RNA, Small Interfering; Tetradecanoylphorbol Acetate; Tumor Necrosis Factor-alpha

2009
Alpha-synuclein-glucocerebrosidase interactions in pharmacological Gaucher models: a biological link between Gaucher disease and parkinsonism.
    Neurotoxicology, 2009, Volume: 30, Issue:6

    Topics: alpha-Synuclein; Analysis of Variance; Animals; Cell Differentiation; Disease Models, Animal; Dose-Response Relationship, Drug; Gaucher Disease; Gene Expression Regulation; Glial Fibrillary Acidic Protein; Humans; Inositol; Mice; Mice, Inbred C57BL; Neuroblastoma; RNA, Messenger; Substantia Nigra

2009
Characterization of Gaucher disease bone marrow mesenchymal stromal cells reveals an altered inflammatory secretome.
    Blood, 2009, Oct-08, Volume: 114, Issue:15

    Topics: Adipocytes; Amino Acid Substitution; Bone Marrow Cells; Cell Differentiation; Cells, Cultured; Chemokine CCL2; Cholesterol; Cyclooxygenase 2; Dinoprostone; Enzyme Inhibitors; Female; Gaucher Disease; Glucosylceramidase; Glucosylceramides; Humans; Inflammation Mediators; Inositol; Interleukin-8; Lysosomes; Male; Middle Aged; Mutation, Missense; Osteocytes; Stromal Cells; Up-Regulation

2009
Uncoupling between CD1d upregulation induced by retinoic acid and conduritol-B-epoxide and iNKT cell responsiveness.
    Immunobiology, 2010, Volume: 215, Issue:6

    Topics: Antigens, CD1d; Antineoplastic Agents; CD4-Positive T-Lymphocytes; CD8-Positive T-Lymphocytes; Cell Division; Cell Line; Chronic Disease; Coculture Techniques; Enzyme Inhibitors; Gaucher Disease; Globosides; Hexosaminidases; Histocompatibility Antigens Class II; Humans; Inflammation; Inositol; Monocytes; Natural Killer T-Cells; Protein Multimerization; RNA, Messenger; Tretinoin; Trihexosylceramides; Up-Regulation

2010
Glucocerebrosidase deficiency dramatically impairs human bone marrow haematopoiesis in an in vitro model of Gaucher disease.
    British journal of haematology, 2010, Volume: 150, Issue:1

    Topics: Bone Marrow Cells; Cell Proliferation; Cells, Cultured; Colony-Forming Units Assay; Enzyme Inhibitors; Flow Cytometry; Gaucher Disease; Glucosylceramidase; Hematopoiesis; Hematopoietic Stem Cells; Humans; Inositol; Models, Biological; Tissue Culture Techniques

2010
Accumulation and distribution of α-synuclein and ubiquitin in the CNS of Gaucher disease mouse models.
    Molecular genetics and metabolism, 2011, Volume: 102, Issue:4

    Topics: Age Factors; alpha-Synuclein; Animals; beta-Glucosidase; Brain; Disease Models, Animal; Gaucher Disease; Glucosylceramides; Inclusion Bodies; Inositol; Mice; Mutation, Missense; Phenotype; Psychosine; Ubiquitin

2011
Alpha-synuclein interacts with Glucocerebrosidase providing a molecular link between Parkinson and Gaucher diseases.
    The Journal of biological chemistry, 2011, Aug-12, Volume: 286, Issue:32

    Topics: alpha-Synuclein; Amino Acid Substitution; Cell Line, Tumor; Enzyme Inhibitors; Gaucher Disease; Glucosylceramidase; Humans; Hydrogen-Ion Concentration; Inositol; Lysosomes; Multiprotein Complexes; Mutation, Missense; Parkinson Disease

2011
Bone marrow microenvironment in an in vitro model of Gaucher disease: consequences of glucocerebrosidase deficiency.
    Stem cells and development, 2012, Jan-20, Volume: 21, Issue:2

    Topics: Bone Marrow; Bone Resorption; Cell Cycle; Cell Differentiation; Cell Movement; Cell Proliferation; Cells, Cultured; Cellular Microenvironment; Culture Media, Conditioned; Gaucher Disease; Glucosylceramidase; Humans; Inositol; Mesenchymal Stem Cells; Models, Biological; Osteoblasts; Osteoclasts

2012
A reconstructed human epidermal keratinization culture model to characterize ceramide metabolism in the stratum corneum.
    Archives of dermatological research, 2012, Volume: 304, Issue:7

    Topics: Acid Ceramidase; Cell Culture Techniques; Cells, Cultured; Ceramides; Desipramine; Epidermis; Feasibility Studies; Gene Expression Regulation; Glucosylceramidase; Glucosyltransferases; Granulocyte-Macrophage Colony-Stimulating Factor; Humans; Inositol; Interleukin-1alpha; Keratinocytes; Models, Biological; Morpholines; Sphingolipids; Sphingomyelin Phosphodiesterase; Tretinoin

2012
Glucocerebrosidase inhibition causes mitochondrial dysfunction and free radical damage.
    Neurochemistry international, 2013, Volume: 62, Issue:1

    Topics: Adenosine Diphosphate; Adenosine Triphosphate; Blotting, Western; Cell Line; Electron Transport; Enzyme Inhibitors; Free Radicals; Glucosylceramidase; Humans; Indicators and Reagents; Inositol; Lysosomes; Membrane Potential, Mitochondrial; Mitochondria; Oxidative Stress; Phosphorylation; Proteasome Endopeptidase Complex; Ubiquitin

2013
Loss of β-glucocerebrosidase activity does not affect alpha-synuclein levels or lysosomal function in neuronal cells.
    PloS one, 2013, Volume: 8, Issue:4

    Topics: alpha-Synuclein; Animals; Autophagy; Cell Differentiation; Cell Line; Gaucher Disease; Glucosylceramidase; Humans; Inositol; Lysosomes; Neurons; Parkinson Disease; Protein Multimerization; Rats

2013
β-Glucosidase 2 (GBA2) activity and imino sugar pharmacology.
    The Journal of biological chemistry, 2013, Sep-06, Volume: 288, Issue:36

    Topics: 1-Deoxynojirimycin; Animals; beta-Glucosidase; Cell Line, Tumor; Cerebellar Ataxia; Chlorocebus aethiops; COS Cells; Enzyme Inhibitors; Glucosylceramidase; Humans; Hydrogen-Ion Concentration; Inositol; Mice; Spastic Paraplegia, Hereditary

2013
Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease.
    Gene, 2013, Dec-15, Volume: 532, Issue:2

    Topics: Animals; Antigens, Differentiation; Bone Marrow Cells; Calcification, Physiologic; Cell Differentiation; Cells, Cultured; Culture Media, Conditioned; Gaucher Disease; Inositol; Mice; Mice, Inbred C57BL; Mice, Knockout; Osteoblasts; Osteoclasts; Osteolysis; Tumor Necrosis Factor-alpha

2013
Neuroinflammation and α-synuclein accumulation in response to glucocerebrosidase deficiency are accompanied by synaptic dysfunction.
    Molecular genetics and metabolism, 2014, Volume: 111, Issue:2

    Topics: alpha-Synuclein; Animals; Corpus Striatum; Disease Models, Animal; Dopamine; Evoked Potentials, Motor; Female; Gaucher Disease; Glucosylceramidase; Humans; Inflammation; Inositol; Male; Mice; MicroRNAs; Mutation; Neuronal Plasticity; Parkinson Disease; Synapses; Synaptic Transmission

2014
Multiple pathogenic proteins implicated in neuronopathic Gaucher disease mice.
    Human molecular genetics, 2014, Aug-01, Volume: 23, Issue:15

    Topics: alpha-Synuclein; Amyloid beta-Protein Precursor; Animals; beta-Glucosidase; Cells, Cultured; Cerebral Cortex; Corpus Striatum; Disease Models, Animal; Enzyme Inhibitors; Gaucher Disease; Gene Expression Regulation; Hippocampus; Humans; Inositol; Lysosomal Membrane Proteins; Membrane Transport Proteins; Mice; Microtubule-Associated Proteins; Mitochondria; Mitochondrial Proteins; Neurons; Prostaglandin-Endoperoxide Synthases; Protein Aggregation, Pathological; Substantia Nigra

2014
Sustained Systemic Glucocerebrosidase Inhibition Induces Brain α-Synuclein Aggregation, Microglia and Complement C1q Activation in Mice.
    Antioxidants & redox signaling, 2015, Aug-20, Volume: 23, Issue:6

    Topics: alpha-Synuclein; Animals; Autophagy; Axonal Transport; Cerebral Cortex; Complement Activation; Complement C1q; Glucosylceramidase; Inositol; Male; Mice; Microglia; Parkinson Disease, Secondary; Protein Aggregation, Pathological; Proteins; Synaptic Transmission

2015
Glucocerebrosidase deficiency and mitochondrial impairment in experimental Parkinson disease.
    Journal of the neurological sciences, 2015, Sep-15, Volume: 356, Issue:1-2

    Topics: 1-Deoxynojirimycin; 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine; Animals; Brain; Cell Count; Cells, Cultured; Disease Models, Animal; Embryo, Mammalian; Gaucher Disease; Glycoside Hydrolase Inhibitors; Histocompatibility Antigens; Inositol; L-Lactate Dehydrogenase; Male; Mesencephalon; Mice; Mice, Inbred C57BL; Mitochondrial Diseases; Neurons; Parkinsonian Disorders; Tyrosine 3-Monooxygenase

2015
CNS-accessible Inhibitor of Glucosylceramide Synthase for Substrate Reduction Therapy of Neuronopathic Gaucher Disease.
    Molecular therapy : the journal of the American Society of Gene Therapy, 2016, Volume: 24, Issue:6

    Topics: Administration, Oral; Animals; Carbamates; Central Nervous System; Disease Models, Animal; Enzyme Inhibitors; Gaucher Disease; Glucosyltransferases; Glycolipids; Humans; Inositol; Liver; Lysosomes; Mice; Quinuclidines; Tissue Distribution; Treatment Outcome

2016
Glucosyl epi-cyclophellitol allows mechanism-based inactivation and structural analysis of human pancreatic α-amylase.
    FEBS letters, 2016, Volume: 590, Issue:8

    Topics: Catalytic Domain; Computer Simulation; Cyclohexanols; Disaccharides; Epoxy Compounds; Humans; Hydrogen Bonding; Inositol; Kinetics; Mass Spectrometry; Models, Molecular; Pancreatic alpha-Amylases; Water; X-Ray Diffraction

2016
Delineating pathological pathways in a chemically induced mouse model of Gaucher disease.
    The Journal of pathology, 2016, Volume: 239, Issue:4

    Topics: Animals; Disease Models, Animal; Gaucher Disease; Gene Expression Profiling; Inositol; Mice

2016
Identification of Modifier Genes in a Mouse Model of Gaucher Disease.
    Cell reports, 2016, 09-06, Volume: 16, Issue:10

    Topics: Animals; Base Sequence; Disease Models, Animal; Gaucher Disease; Genes, Modifier; Genome-Wide Association Study; Injections; Inositol; Longevity; Mice, Inbred Strains; Phenotype; Polymorphism, Single Nucleotide; Receptors, N-Methyl-D-Aspartate

2016
Osteocyte Alterations Induce Osteoclastogenesis in an In Vitro Model of Gaucher Disease.
    International journal of molecular sciences, 2017, Jan-13, Volume: 18, Issue:1

    Topics: Animals; Apoptosis; Bone Marrow Cells; Cell Differentiation; Cell Line; Connexin 43; Culture Media, Conditioned; Female; Gaucher Disease; Inositol; Integrin beta Chains; Interleukin-6; Macrophages; Mice, Inbred C57BL; Models, Biological; Osteoclasts; Osteocytes; Osteogenesis; Osteoprotegerin; RANK Ligand; Solubility

2017
Inhibition of neuronal mitochondrial complex I or lysosomal glucocerebrosidase is associated with increased dopamine and serotonin turnover.
    Neurochemistry international, 2017, Volume: 109

    Topics: Cell Line, Tumor; Dopamine; Electron Transport Complex I; Enzyme Inhibitors; Glucosylceramidase; Humans; Inositol; Lysosomes; Mitochondria; Neurons; Serotonin

2017
Modulation of β-glucocerebrosidase increases α-synuclein secretion and exosome release in mouse models of Parkinson's disease.
    Human molecular genetics, 2018, 05-15, Volume: 27, Issue:10

    Topics: alpha-Synuclein; Animals; Brain; Disease Models, Animal; Exosomes; Glucosylceramidase; Humans; Inositol; Lysosomes; Mice; Mutation; Parkinson Disease

2018
Development and biochemical characterization of a mouse model of Parkinson's disease bearing defective glucocerebrosidase activity.
    Neurobiology of disease, 2019, Volume: 124

    Topics: Animals; Disease Models, Animal; Enzyme Inhibitors; Glucosylceramidase; Inositol; Male; Mice; Mice, Inbred C57BL; Parkinsonian Disorders

2019
In vivo inactivation of glycosidases by conduritol B epoxide and cyclophellitol as revealed by activity-based protein profiling.
    The FEBS journal, 2019, Volume: 286, Issue:3

    Topics: Animals; beta-Glucosidase; Brain; Cyclohexanols; Disease Models, Animal; Enzyme Assays; Glucosylceramidase; Glycoside Hydrolase Inhibitors; HEK293 Cells; Humans; Inositol; Isoenzymes; Kinetics; Larva; Lysosomes; Mice; Parkinson Disease; Zebrafish

2019
GBA RNAi but not catalytic inhibition of glucocerebrosidase with Conduritol-β-epoxide increases levels of total α-synuclein in SH-SY5Y cells.
    Neuroscience letters, 2019, 07-27, Volume: 706

    Topics: alpha-Synuclein; Animals; Cell Line, Tumor; Cerebral Cortex; Glucosylceramidase; Humans; Inositol; Neurons; Rats; Rats, Wistar; RNA Interference

2019
Chemical inhibition of β-glucocerebrosidase does not affect phagocytosis and early containment of Leishmania by murine macrophages.
    Experimental parasitology, 2020, Volume: 216

    Topics: Animals; Enzyme Inhibitors; Flow Cytometry; Gaucher Disease; Glucosylceramidase; Inositol; Leishmania; Leishmania infantum; Leishmania major; Leishmania mexicana; Lysosomes; Macrophages; Mice; Mice, Inbred C57BL; Microscopy, Fluorescence; Phagocytosis

2020
Pathological α-syn aggregation is mediated by glycosphingolipid chain length and the physiological state of α-syn in vivo.
    Proceedings of the National Academy of Sciences of the United States of America, 2021, 12-14, Volume: 118, Issue:50

    Topics: alpha-Synuclein; Amino Acid Sequence; Animals; Brain; Cathepsins; Cell Differentiation; Glycosphingolipids; Humans; Induced Pluripotent Stem Cells; Inositol; Lysosomes; Mice; Time Factors

2021
Seventy-Two-Hour LRRK2 Kinase Activity Inhibition Increases Lysosomal GBA Expression in H4, a Human Neuroglioma Cell Line.
    International journal of molecular sciences, 2022, Jun-22, Volume: 23, Issue:13

    Topics: Cell Line; Cell Line, Tumor; Enzyme Inhibitors; Glioma; Glucosylceramidase; Humans; Inositol; Leucine-Rich Repeat Serine-Threonine Protein Kinase-2; Lysosomes; Mutation; Parkinson Disease

2022