178 related articles for article (PubMed ID: 22033823)
1. A high throughput glucocerebrosidase assay using the natural substrate glucosylceramide.
Motabar O; Goldin E; Leister W; Liu K; Southall N; Huang W; Marugan JJ; Sidransky E; Zheng W
Anal Bioanal Chem; 2012 Jan; 402(2):731-9. PubMed ID: 22033823
[TBL] [Abstract][Full Text] [Related]
2. Optimization and validation of two miniaturized glucocerebrosidase enzyme assays for high throughput screening.
Urban DJ; Zheng W; Goker-Alpan O; Jadhav A; Lamarca ME; Inglese J; Sidransky E; Austin CP
Comb Chem High Throughput Screen; 2008 Dec; 11(10):817-24. PubMed ID: 19075603
[TBL] [Abstract][Full Text] [Related]
3. A New Glucocerebrosidase Chaperone Reduces α-Synuclein and Glycolipid Levels in iPSC-Derived Dopaminergic Neurons from Patients with Gaucher Disease and Parkinsonism.
Aflaki E; Borger DK; Moaven N; Stubblefield BK; Rogers SA; Patnaik S; Schoenen FJ; Westbroek W; Zheng W; Sullivan P; Fujiwara H; Sidhu R; Khaliq ZM; Lopez GJ; Goldstein DS; Ory DS; Marugan J; Sidransky E
J Neurosci; 2016 Jul; 36(28):7441-52. PubMed ID: 27413154
[TBL] [Abstract][Full Text] [Related]
4. Progress and potential of non-inhibitory small molecule chaperones for the treatment of Gaucher disease and its implications for Parkinson disease.
Jung O; Patnaik S; Marugan J; Sidransky E; Westbroek W
Expert Rev Proteomics; 2016 May; 13(5):471-9. PubMed ID: 27098312
[TBL] [Abstract][Full Text] [Related]
5. Improved management of lysosomal glucosylceramide levels in a mouse model of type 1 Gaucher disease using enzyme and substrate reduction therapy.
Marshall J; McEachern KA; Chuang WL; Hutto E; Siegel CS; Shayman JA; Grabowski GA; Scheule RK; Copeland DP; Cheng SH
J Inherit Metab Dis; 2010 Jun; 33(3):281-9. PubMed ID: 20336375
[TBL] [Abstract][Full Text] [Related]
6. Clinical phenotype of Gaucher disease in relation to properties of mutant glucocerebrosidase in cultured fibroblasts.
Van Weely S; Van Leeuwen MB; Jansen ID; De Bruijn MA; Brouwer-Kelder EM; Schram AW; Sa Miranda MC; Barranger JA; Petersen EM; Goldblatt J
Biochim Biophys Acta; 1991 Jun; 1096(4):301-11. PubMed ID: 1829642
[TBL] [Abstract][Full Text] [Related]
7. Comparison of synthetic and natural glucosylceramides as substrate for glucosylceramidase assay.
Vaccaro AM; Kobayashi T; Suzuki K
Clin Chim Acta; 1982 Jan; 118(1):1-7. PubMed ID: 7053901
[TBL] [Abstract][Full Text] [Related]
8. An evolutionary and structure-based docking model for glucocerebrosidase-saposin C and glucocerebrosidase-substrate interactions - relevance for Gaucher disease.
Atrian S; López-Viñas E; Gómez-Puertas P; Chabás A; Vilageliu L; Grinberg D
Proteins; 2008 Feb; 70(3):882-91. PubMed ID: 17803231
[TBL] [Abstract][Full Text] [Related]
9. High throughput screening for small molecule therapy for Gaucher disease using patient tissue as the source of mutant glucocerebrosidase.
Goldin E; Zheng W; Motabar O; Southall N; Choi JH; Marugan J; Austin CP; Sidransky E
PLoS One; 2012; 7(1):e29861. PubMed ID: 22272254
[TBL] [Abstract][Full Text] [Related]
10. Fluorescence-Quenched Substrates for Quantitative Live Cell Imaging of Glucocerebrosidase Activity.
Ashmus RA; Shen DL; Vocadlo DJ
Methods Enzymol; 2018; 598():199-215. PubMed ID: 29306435
[TBL] [Abstract][Full Text] [Related]
11. Analysis of glucocerebrosidase activity using N-(1-[14C]hexanoyl)-D-erythroglucosylsphingosine demonstrates a correlation between levels of residual enzyme activity and the type of Gaucher disease.
Meivar-Levy I; Horowitz M; Futerman AH
Biochem J; 1994 Oct; 303 ( Pt 2)(Pt 2):377-82. PubMed ID: 7980395
[TBL] [Abstract][Full Text] [Related]
12. Comparative study on glucocerebrosidase in spleens from patients with Gaucher disease.
Aerts JM; Donker-Koopman WE; Brul S; Van Weely S; Sa Miranda MC; Barranger JA; Tager JM; Schram AW
Biochem J; 1990 Jul; 269(1):93-100. PubMed ID: 2198026
[TBL] [Abstract][Full Text] [Related]
13. Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage.
Mizukami H; Mi Y; Wada R; Kono M; Yamashita T; Liu Y; Werth N; Sandhoff R; Sandhoff K; Proia RL
J Clin Invest; 2002 May; 109(9):1215-21. PubMed ID: 11994410
[TBL] [Abstract][Full Text] [Related]
14. Gaucher disease. III. Substrate specificity of glucocerebrosidase and the use of nonlabeled natural substrates for the investigation of patients.
Choy FY; Davidson RG
Am J Hum Genet; 1980 Sep; 32(5):670-80. PubMed ID: 6775530
[TBL] [Abstract][Full Text] [Related]
15. A new glucocerebrosidase-deficient neuronal cell model provides a tool to probe pathophysiology and therapeutics for Gaucher disease.
Westbroek W; Nguyen M; Siebert M; Lindstrom T; Burnett RA; Aflaki E; Jung O; Tamargo R; Rodriguez-Gil JL; Acosta W; Hendrix A; Behre B; Tayebi N; Fujiwara H; Sidhu R; Renvoise B; Ginns EI; Dutra A; Pak E; Cramer C; Ory DS; Pavan WJ; Sidransky E
Dis Model Mech; 2016 Jul; 9(7):769-78. PubMed ID: 27482815
[TBL] [Abstract][Full Text] [Related]
16. Glucosylceramide mimics: highly potent GCase inhibitors and selective pharmacological chaperones for mutations associated with types 1 and 2 Gaucher disease.
Schönemann W; Gallienne E; Ikeda-Obatake K; Asano N; Nakagawa S; Kato A; Adachi I; Górecki M; Frelek J; Martin OR
ChemMedChem; 2013 Nov; 8(11):1805-17. PubMed ID: 24115322
[TBL] [Abstract][Full Text] [Related]
17. Intracellular degradation of fluorescent glycolipids by lysosomal enzymes and their activators.
Madar-Shapiro L; Pasmanik-Chor M; Dinur T; Dagan A; Gatt S; Horowitz M
J Inherit Metab Dis; 1999 Jun; 22(5):623-37. PubMed ID: 10399095
[TBL] [Abstract][Full Text] [Related]
18. Neuronal accumulation of glucosylceramide in a mouse model of neuronopathic Gaucher disease leads to neurodegeneration.
Farfel-Becker T; Vitner EB; Kelly SL; Bame JR; Duan J; Shinder V; Merrill AH; Dobrenis K; Futerman AH
Hum Mol Genet; 2014 Feb; 23(4):843-54. PubMed ID: 24064337
[TBL] [Abstract][Full Text] [Related]
19. Accumulation of protein-bound epidermal glucosylceramides in beta-glucocerebrosidase deficient type 2 Gaucher mice.
Doering T; Proia RL; Sandhoff K
FEBS Lett; 1999 Mar; 447(2-3):167-70. PubMed ID: 10214939
[TBL] [Abstract][Full Text] [Related]
20. Hydrophilic iminosugar active-site-specific chaperones increase residual glucocerebrosidase activity in fibroblasts from Gaucher patients.
Chang HH; Asano N; Ishii S; Ichikawa Y; Fan JQ
FEBS J; 2006 Sep; 273(17):4082-92. PubMed ID: 16934036
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
