conduritol epoxide has been researched along with Acid beta-Glucosidase Deficiency in 43 studies
conduritol epoxide: conduritol C epoxide refers to the (epi & neo)-isomers; structure
conduritol epoxide : An epoxide resulting from the epoxidation of the double bond of a conduritol.
| Excerpt | Relevance | Reference |
|---|---|---|
| "To elucidate the genetic heterogeneity in Gaucher disease, the residual beta-glucosidase in cultured fibroblasts from affected patients with each of the major phenotypes was investigated in vitro and/or in viable cells by inhibitor studies using the covalent catalytic site inhibitors, conduritol B epoxide or its bromo derivative, and the reversible cationic inhibitor, sphingosine." | 7.67 | Gaucher disease types 1, 2, and 3: differential mutations of the acid beta-glucosidase active site identified with conduritol B epoxide derivatives and sphingosine. ( Dinur, T; Gatt, S; Grabowski, GA; Kruse, JR; Legler, G; Osiecki, KM, 1985) |
| "To elucidate the genetic heterogeneity in Gaucher disease, the residual beta-glucosidase in cultured fibroblasts from affected patients with each of the major phenotypes was investigated in vitro and/or in viable cells by inhibitor studies using the covalent catalytic site inhibitors, conduritol B epoxide or its bromo derivative, and the reversible cationic inhibitor, sphingosine." | 3.67 | Gaucher disease types 1, 2, and 3: differential mutations of the acid beta-glucosidase active site identified with conduritol B epoxide derivatives and sphingosine. ( Dinur, T; Gatt, S; Grabowski, GA; Kruse, JR; Legler, G; Osiecki, KM, 1985) |
| "Gaucher disease is a lysosomal storage disease in which a genetic deficiency in β-glucocerebrosidase leads to the accumulation of glycosphingolipids in lysosomes." | 1.56 | Chemical inhibition of β-glucocerebrosidase does not affect phagocytosis and early containment of Leishmania by murine macrophages. ( Castro, H; Macedo, MF; Ribeiro, H; Rocha, MI, 2020) |
| "Gaucher disease is an autosomal recessive disease, caused by a lack or functional deficiency of the lysosomal enzyme, glucocerebrosidase (GCase)." | 1.42 | Glucocerebrosidase deficiency and mitochondrial impairment in experimental Parkinson disease. ( Alvarez-Fischer, D; Andreas, H; Hirsch, EC; Höglinger, GU; Höllerhage, M; Lu, L; Noelker, C; Oertel, WH; Roscher, R; Sturn, A; Vulinovic, F, 2015) |
| "One of the cardinal symptoms of type 1 Gaucher Disease (GD) is cytopenia, usually explained by bone marrow (BM) infiltration by Gaucher cells and hypersplenism." | 1.36 | Glucocerebrosidase deficiency dramatically impairs human bone marrow haematopoiesis in an in vitro model of Gaucher disease. ( Berger, J; Berger, MG; Boiret-Dupré, N; Boisgard, S; Caillaud, C; Chomienne, C; Larghero, J; Lecourt, S; Marolleau, JP; Rapatel, C; Vanneaux, V, 2010) |
| "Gaucher disease is an inherited metabolic disorder caused by mutations in the lysosomal enzyme acid-beta-glucosidase (GlcCerase)." | 1.33 | X-ray structure of human acid-beta-glucosidase covalently bound to conduritol-B-epoxide. Implications for Gaucher disease. ( Boldin-Adamsky, S; Futerman, AH; Kelly, JW; Premkumar, L; Sawkar, AR; Silman, I; Sussman, JL; Toker, L, 2005) |
| "Gaucher disease is the most common lysosomal glycosphingolipid storage disease; decreased activity of glucosylceramide beta-glucosidase (GCase) results in the accumulation of glucocerebroside (GlcCer) in macrophage-derived cells." | 1.29 | In vitro accumulation of glucocerebroside in neuroblastoma cells: a model for study of Gaucher disease pathobiology. ( Chaturvedi, P; Newburg, DS; Prence, EM, 1996) |
| "To date, enzymatic diagnosis of Gaucher's disease via a fluorometric assay procedure which utilizes 4-methylumbelliferyl-beta-D-glucopyranoside as a substrate has not been possible when liver serves as the source of enzyme since currently employed fluorometric procedures cannot adequately differentiate between a broad-specificity beta-glucosidase and lysosomal glucocerebrosidase activities in crude extracts of liver." | 1.26 | A revised fluorometric assay for Gaucher's disease using conduritol-beta-epoxide with liver as the source of Beta-glucosidase. ( Daniels, LB; Glew, RH; Radin, NS; Vunnam, RR, 1980) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 17 (39.53) | 18.7374 |
| 1990's | 3 (6.98) | 18.2507 |
| 2000's | 7 (16.28) | 29.6817 |
| 2010's | 15 (34.88) | 24.3611 |
| 2020's | 1 (2.33) | 2.80 |
| Authors | Studies |
|---|---|
| Ribeiro, H | 1 |
| Rocha, MI | 1 |
| Castro, H | 1 |
| Macedo, MF | 1 |
| Dermentzaki, G | 1 |
| Dimitriou, E | 1 |
| Xilouri, M | 1 |
| Michelakakis, H | 1 |
| Stefanis, L | 1 |
| Mucci, JM | 2 |
| Suqueli García, F | 1 |
| de Francesco, PN | 1 |
| Ceci, R | 1 |
| Di Genaro, S | 1 |
| Fossati, CA | 1 |
| Delpino, MV | 2 |
| Rozenfeld, PA | 2 |
| Ginns, EI | 1 |
| Mak, SK | 1 |
| Ko, N | 1 |
| Karlgren, J | 1 |
| Akbarian, S | 1 |
| Chou, VP | 1 |
| Guo, Y | 1 |
| Lim, A | 1 |
| Samuelsson, S | 1 |
| LaMarca, ML | 1 |
| Vazquez-DeRose, J | 1 |
| Manning-Boğ, AB | 2 |
| Xu, YH | 3 |
| Xu, K | 1 |
| Sun, Y | 4 |
| Liou, B | 1 |
| Quinn, B | 3 |
| Li, RH | 1 |
| Xue, L | 1 |
| Zhang, W | 1 |
| Setchell, KD | 1 |
| Witte, D | 3 |
| Grabowski, GA | 8 |
| Noelker, C | 1 |
| Lu, L | 1 |
| Höllerhage, M | 1 |
| Vulinovic, F | 1 |
| Sturn, A | 1 |
| Roscher, R | 1 |
| Höglinger, GU | 1 |
| Hirsch, EC | 1 |
| Oertel, WH | 1 |
| Alvarez-Fischer, D | 1 |
| Andreas, H | 1 |
| Marshall, J | 1 |
| Bangari, DS | 1 |
| Budman, E | 1 |
| Park, H | 1 |
| Nietupski, JB | 1 |
| Allaire, A | 1 |
| Cromwell, MA | 1 |
| Wang, B | 1 |
| Leonard, JP | 1 |
| Cheng, SH | 1 |
| Hayashi, Y | 1 |
| Ito, M | 1 |
| Vardi, A | 1 |
| Zigdon, H | 1 |
| Meshcheriakova, A | 1 |
| Klein, AD | 2 |
| Yaacobi, C | 1 |
| Eilam, R | 1 |
| Kenwood, BM | 1 |
| Rahim, AA | 1 |
| Massaro, G | 1 |
| Merrill, AH | 2 |
| Vitner, EB | 1 |
| Futerman, AH | 5 |
| Ferreira, NS | 1 |
| Ben-Dor, S | 1 |
| Duan, J | 1 |
| Hardy, J | 1 |
| Cox, TM | 1 |
| Bondar, C | 1 |
| Ormazabal, M | 1 |
| Crivaro, A | 1 |
| Ferreyra-Compagnucci, M | 1 |
| Schüle, B | 1 |
| Langston, JW | 1 |
| Campeau, PM | 1 |
| Rafei, M | 1 |
| Boivin, MN | 1 |
| Galipeau, J | 1 |
| Balreira, A | 1 |
| Cavallari, M | 1 |
| Sá Miranda, MC | 2 |
| Arosa, FA | 1 |
| Berger, J | 1 |
| Lecourt, S | 2 |
| Vanneaux, V | 2 |
| Rapatel, C | 1 |
| Boisgard, S | 1 |
| Caillaud, C | 2 |
| Boiret-Dupré, N | 1 |
| Chomienne, C | 2 |
| Marolleau, JP | 2 |
| Larghero, J | 2 |
| Berger, MG | 1 |
| Ran, H | 1 |
| Yap, TL | 1 |
| Gruschus, JM | 1 |
| Velayati, A | 1 |
| Westbroek, W | 1 |
| Goldin, E | 1 |
| Moaven, N | 1 |
| Sidransky, E | 1 |
| Lee, JC | 1 |
| Cras, A | 1 |
| Freida, D | 1 |
| Heraoui, D | 1 |
| Herbi, L | 1 |
| Belmatoug, N | 1 |
| Bodennec, J | 1 |
| Pelled, D | 2 |
| Riebeling, C | 1 |
| Trajkovic, S | 1 |
| Schueler, UH | 1 |
| Kolter, T | 1 |
| Kaneski, CR | 1 |
| Zirzow, GC | 1 |
| Sandhoff, K | 1 |
| Brady, RO | 1 |
| Premkumar, L | 1 |
| Sawkar, AR | 1 |
| Boldin-Adamsky, S | 1 |
| Toker, L | 1 |
| Silman, I | 1 |
| Kelly, JW | 1 |
| Sussman, JL | 1 |
| Reboulet, R | 1 |
| Huelsken, J | 1 |
| Daniels, LB | 2 |
| Glew, RH | 4 |
| Radin, NS | 2 |
| Vunnam, RR | 1 |
| Stephens, MC | 3 |
| Bernatsky, A | 2 |
| Singh, H | 2 |
| Kanfer, JN | 3 |
| Legler, G | 6 |
| Diven, WF | 1 |
| Lee, RE | 2 |
| Gatt, S | 5 |
| Dinur, T | 4 |
| Desnick, RJ | 3 |
| Gregoriadis, G | 1 |
| Weereratne, H | 1 |
| Blair, H | 1 |
| Bull, GM | 1 |
| Kohen, E | 1 |
| Kohen, C | 1 |
| Hirschberg, JG | 1 |
| Santus, R | 1 |
| Grabowski, G | 1 |
| Mangel, W | 1 |
| Prince, J | 1 |
| van Weely, S | 1 |
| Brandsma, M | 1 |
| Strijland, A | 1 |
| Tager, JM | 3 |
| Aerts, JM | 3 |
| Prence, EM | 1 |
| Chaturvedi, P | 1 |
| Newburg, DS | 4 |
| Shogomori, H | 1 |
| Burachinsky, V | 1 |
| Butcher, BA | 1 |
| Gopalan, V | 1 |
| Richards, TC | 1 |
| Waggoner, AS | 1 |
| Donker-Koopman, WE | 1 |
| Koot, M | 1 |
| Barranger, JA | 2 |
| Schram, AW | 2 |
| Yatziv, S | 3 |
| McCluer, RH | 2 |
| Raghavan, S | 1 |
| Osiecki-Newman, K | 1 |
| Fabbro, D | 1 |
| Pinto, RA | 1 |
| Magalhaes, JA | 1 |
| Livni, N | 1 |
| Barfi, G | 1 |
| Kolodny, EH | 1 |
| Shea, TB | 1 |
| Raghavan, SS | 1 |
| Osiecki, KM | 2 |
| Basu, A | 1 |
| Kruse, JR | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Ambroxol as a Novel Disease Modifying Treatment for Lewy Body Dementia[NCT04405596] | Phase 1/Phase 2 | 15 participants (Anticipated) | Interventional | 2025-01-31 | Not yet recruiting | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
1 review available for conduritol epoxide and Acid beta-Glucosidase Deficiency
| Article | Year |
|---|---|
Klotho-Related Protein KLrP: Structure and Functions.
Topics: Animals; Brain; Crystallography, X-Ray; Cytosol; Fibroblasts; Gaucher Disease; Gene Knockdown Techni | 2016 |
42 other studies available for conduritol epoxide and Acid beta-Glucosidase Deficiency
| Article | Year |
|---|---|
Chemical inhibition of β-glucocerebrosidase does not affect phagocytosis and early containment of Leishmania by murine macrophages.
Topics: Animals; Enzyme Inhibitors; Flow Cytometry; Gaucher Disease; Glucosylceramidase; Inositol; Leishmani | 2020 |
Loss of β-glucocerebrosidase activity does not affect alpha-synuclein levels or lysosomal function in neuronal cells.
Topics: alpha-Synuclein; Animals; Autophagy; Cell Differentiation; Cell Line; Gaucher Disease; Glucosylceram | 2013 |
Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease.
Topics: Animals; Antigens, Differentiation; Bone Marrow Cells; Calcification, Physiologic; Cell Differentiat | 2013 |
Neuroinflammation and α-synuclein accumulation in response to glucocerebrosidase deficiency are accompanied by synaptic dysfunction.
Topics: alpha-Synuclein; Animals; Corpus Striatum; Disease Models, Animal; Dopamine; Evoked Potentials, Moto | 2014 |
Multiple pathogenic proteins implicated in neuronopathic Gaucher disease mice.
Topics: alpha-Synuclein; Amyloid beta-Protein Precursor; Animals; beta-Glucosidase; Cells, Cultured; Cerebra | 2014 |
Glucocerebrosidase deficiency and mitochondrial impairment in experimental Parkinson disease.
Topics: 1-Deoxynojirimycin; 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine; Animals; Brain; Cell Count; Cells, | 2015 |
CNS-accessible Inhibitor of Glucosylceramide Synthase for Substrate Reduction Therapy of Neuronopathic Gaucher Disease.
Topics: Administration, Oral; Animals; Carbamates; Central Nervous System; Disease Models, Animal; Enzyme In | 2016 |
Delineating pathological pathways in a chemically induced mouse model of Gaucher disease.
Topics: Animals; Disease Models, Animal; Gaucher Disease; Gene Expression Profiling; Inositol; Mice | 2016 |
Identification of Modifier Genes in a Mouse Model of Gaucher Disease.
Topics: Animals; Base Sequence; Disease Models, Animal; Gaucher Disease; Genes, Modifier; Genome-Wide Associ | 2016 |
Osteocyte Alterations Induce Osteoclastogenesis in an In Vitro Model of Gaucher Disease.
Topics: Animals; Apoptosis; Bone Marrow Cells; Cell Differentiation; Cell Line; Connexin 43; Culture Media, | 2017 |
Alpha-synuclein-glucocerebrosidase interactions in pharmacological Gaucher models: a biological link between Gaucher disease and parkinsonism.
Topics: alpha-Synuclein; Analysis of Variance; Animals; Cell Differentiation; Disease Models, Animal; Dose-R | 2009 |
Characterization of Gaucher disease bone marrow mesenchymal stromal cells reveals an altered inflammatory secretome.
Topics: Adipocytes; Amino Acid Substitution; Bone Marrow Cells; Cell Differentiation; Cells, Cultured; Chemo | 2009 |
Uncoupling between CD1d upregulation induced by retinoic acid and conduritol-B-epoxide and iNKT cell responsiveness.
Topics: Antigens, CD1d; Antineoplastic Agents; CD4-Positive T-Lymphocytes; CD8-Positive T-Lymphocytes; Cell | 2010 |
Glucocerebrosidase deficiency dramatically impairs human bone marrow haematopoiesis in an in vitro model of Gaucher disease.
Topics: Bone Marrow Cells; Cell Proliferation; Cells, Cultured; Colony-Forming Units Assay; Enzyme Inhibitor | 2010 |
Accumulation and distribution of α-synuclein and ubiquitin in the CNS of Gaucher disease mouse models.
Topics: Age Factors; alpha-Synuclein; Animals; beta-Glucosidase; Brain; Disease Models, Animal; Gaucher Dise | 2011 |
Alpha-synuclein interacts with Glucocerebrosidase providing a molecular link between Parkinson and Gaucher diseases.
Topics: alpha-Synuclein; Amino Acid Substitution; Cell Line, Tumor; Enzyme Inhibitors; Gaucher Disease; Gluc | 2011 |
Bone marrow microenvironment in an in vitro model of Gaucher disease: consequences of glucocerebrosidase deficiency.
Topics: Bone Marrow; Bone Resorption; Cell Cycle; Cell Differentiation; Cell Movement; Cell Proliferation; C | 2012 |
Phosphatidylcholine synthesis is elevated in neuronal models of Gaucher disease due to direct activation of CTP:phosphocholine cytidylyltransferase by glucosylceramide.
Topics: Animals; Axons; Brain; Carbon Radioisotopes; Cell Division; Choline; Choline-Phosphate Cytidylyltran | 2002 |
Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease.
Topics: Animals; Cell Culture Techniques; Cell Line; Chromatography, High Pressure Liquid; Dose-Response Rel | 2004 |
X-ray structure of human acid-beta-glucosidase covalently bound to conduritol-B-epoxide. Implications for Gaucher disease.
Topics: beta-Glucosidase; Binding Sites; Catalysis; Crystallography, X-Ray; Gaucher Disease; Humans; Inosito | 2005 |
Dependence of reversibility and progression of mouse neuronopathic Gaucher disease on acid beta-glucosidase residual activity levels.
Topics: Animals; Central Nervous System; Cytokines; Disease Models, Animal; Disease Progression; Female; Gau | 2008 |
A revised fluorometric assay for Gaucher's disease using conduritol-beta-epoxide with liver as the source of Beta-glucosidase.
Topics: beta-Glucosidase; Clinical Enzyme Tests; Fluorometry; Gaucher Disease; Glucosidases; Glucosylceramid | 1980 |
Distribution of conduritol B epoxide in the animal model for Gaucher's disease (Gaucher mouse).
Topics: Animals; beta-Glucosidase; Blood-Brain Barrier; Disease Models, Animal; Gaucher Disease; Glucosidase | 1981 |
An improved fluorometric leukocyte beta-glucosidase assay for Gaucher's disease.
Topics: Adolescent; beta-Glucosidase; Clinical Enzyme Tests; Female; Fibroblasts; Fluorometry; Gaucher Disea | 1981 |
Studies on human acid beta-glucosidase and the nature of the molecular defect in type 1 Ashkenazi Gaucher disease.
Topics: beta-Glucosidase; Detergents; Europe; Gaucher Disease; Glucosidases; Glucosides; Humans; Hydrolysis; | 1982 |
The Gaucher mouse.
Topics: Acid Phosphatase; Animals; beta-Glucosidase; Brain; Cerebrosides; Disease Models, Animal; Galactosyl | 1982 |
Liposomes in Gaucher type I disease: use in enzyme therapy and the creation of an animal model.
Topics: Adult; Animals; beta-Glucosidase; Disease Models, Animal; Female; Gaucher Disease; Glucosidases; Glu | 1982 |
An in situ study of beta-glucosidase activity in normal and Gaucher fibroblasts with fluorogenic probes.
Topics: beta-Galactosidase; beta-Glucosidase; Cells, Cultured; Fibroblasts; Fluorescent Dyes; Gaucher Diseas | 1993 |
Demonstration of the existence of a second, non-lysosomal glucocerebrosidase that is not deficient in Gaucher disease.
Topics: beta-Glucosidase; Cells, Cultured; Gaucher Disease; Glucosylceramidase; Humans; Hydrogen-Ion Concent | 1993 |
In vitro accumulation of glucocerebroside in neuroblastoma cells: a model for study of Gaucher disease pathobiology.
Topics: alpha-Mannosidase; beta-Galactosidase; beta-Glucosidase; Cell Line; Gaucher Disease; Glucosylceramid | 1996 |
The increased sensitivity of neurons with elevated glucocerebroside to neurotoxic agents can be reversed by imiglucerase.
Topics: Animals; Antimetabolites; Carboxylic Acids; Cell Death; Cells, Cultured; Enzyme Inhibitors; Fumonisi | 2000 |
The Gaucher mouse: differential action of conduritol B epoxide and reversibility of its effects.
Topics: Acetylglucosaminidase; Animals; beta-Glucosidase; Brain; Cerebrosides; Gaucher Disease; Humans; Inos | 1978 |
Use of 4-heptylumbelliferyl-beta-D-glucoside to identify Gaucher's disease heterozygotes.
Topics: beta-Glucosidase; Brain; Gaucher Disease; Genetic Carrier Screening; Glucosylceramidase; Humans; Hyd | 1989 |
Deficient activity of glucocerebrosidase in urine from patients with type 1 Gaucher disease.
Topics: Adolescent; Adult; beta-N-Acetylhexosaminidases; Child; Female; Gaucher Disease; Glucosidases; Gluco | 1986 |
beta-Glucosidase inhibition in murine peritoneal macrophages by conduritol-B-epoxide: an in vitro model of the Gaucher cell.
Topics: Animals; beta-Glucosidase; Cells, Cultured; Dose-Response Relationship, Drug; Gaucher Disease; Gluco | 1986 |
Human acid beta-glucosidase. Use of conduritol B epoxide derivatives to investigate the catalytically active normal and Gaucher disease enzymes.
Topics: beta-Glucosidase; Gaucher Disease; Glucosidases; Humans; Immunoglobulin G; Inositol; Kinetics; Mathe | 1986 |
Heterogeneity in human acid beta-glucosidase revealed by cellulose-acetate electrophoresis.
Topics: beta-Glucosidase; Electrophoresis; Electrophoresis, Cellulose Acetate; Female; Fibroblasts; Gaucher | 1988 |
Gaucher-like changes in human blood-derived macrophages induced by beta-glucocerebrosidase inhibition.
Topics: Blood Cells; Gaucher Disease; Glucosidases; Glucosylceramidase; Humans; Inositol; Macrophages; Micro | 1988 |
Macrophages exposed in vitro to conduritol B epoxide resemble Gaucher cells.
Topics: Animals; Gaucher Disease; Glucosidases; Glucosylceramidase; Glucosylceramides; Inositol; Lysosomes; | 1988 |
Human acid beta-glucosidase: isolation and amino acid sequence of a peptide containing the catalytic site.
Topics: Amino Acid Sequence; Base Sequence; Binding Sites; DNA; Gaucher Disease; Glucosidases; Glucosylceram | 1986 |
Characterization of the activation of rat liver beta-glucosidase by sialosylgangliotetraosylceramide.
Topics: Animals; beta-Glucosidase; Chemical Phenomena; Chemistry, Physical; Dose-Response Relationship, Drug | 1985 |
Gaucher disease types 1, 2, and 3: differential mutations of the acid beta-glucosidase active site identified with conduritol B epoxide derivatives and sphingosine.
Topics: Adolescent; Adult; Binding Sites; Cells, Cultured; Child; Child, Preschool; Female; Fibroblasts; Gau | 1985 |