conduritol epoxide has been researched along with Gaucher Disease 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.
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.
| 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 Gaucher Disease
| 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 Gaucher Disease
| 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 |