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Tailoring the Specificity and Reactivity of a Mechanism-Based Inactivator of Glucocerebrosidase for Potential Therapeutic Applications.
Published in:
Angewandte Chemie, 2011, v. 123, n. 44, p. 10565, doi. 10.1002/ange.201103924
By:
- Rempel, Brian P.;
- Tropak, Michael B.;
- Mahuran, Don J.;
- Withers, Stephen G.
Publication type:
Article
Impaired Glucose Tolerance in a Mouse Model of Sidt2 Deficiency.
Published in:
PLoS ONE, 2013, v. 8, n. 6, p. 1, doi. 10.1371/journal.pone.0066139
By:
- Gao, Jialin;
- Gu, Xuefan;
- Mahuran, Don J.;
- Wang, Zhugang;
- Zhang, Huiwen
Publication type:
Article
In Cellulo Examination of a Beta-Alpha Hybrid Construct of Beta-Hexosaminidase A Subunits, Reported to Interact with the GM2 Activator Protein and Hydrolyze GM2 Ganglioside.
Published in:
PLoS ONE, 2013, v. 8, n. 3, p. 1, doi. 10.1371/journal.pone.0057908
By:
- Sinici, Incilay;
- Yonekawa, Sayuri;
- Tkachyova, Ilona;
- Gray, Steven J.;
- Samulski, R. Jude;
- Wakarchuk, Warren;
- Mark, Brian L.;
- Mahuran, Don J.
Publication type:
Article
Fluorous Iminoalditols: A New Family of Glycosidase Inhibitors and Pharmacological Chaperones.
Published in:
ChemBioChem, 2010, v. 11, n. 14, p. 2026, doi. 10.1002/cbic.201000192
By:
- Schitter, Georg;
- Steiner, Andreas J.;
- Pototschnig, Gerit;
- Scheucher, Elisabeth;
- Thonhofer, Martin;
- Tarling, Chris A.;
- Withers, Stephen G.;
- Fantur, Katrin;
- Paschke, Eduard;
- Mahuran, Don J.;
- Rigat, Brigitte A.;
- Tropak, Michael B.;
- Illaszewicz, Carina;
- Saf, Robert;
- Stütz, Arnold E.;
- Wrodnigg, Tanja M.
Publication type:
Article
MACROAUTOPHAGY-LYSOSOMAL SYSTEM (MALS) IN GAUCHER PATIENTS CARRYING L444P AND N370S MUTATIONS.
Published in:
2012
By:
- LAY, Incilay;
- TKACHYOVA, Ilona;
- TROPAK, Michael B.;
- RIGAT, Brigitte;
- MAHURAN, Don J.
Publication type:
Abstract
L444P ve N370S MUTASYONU TAŞIYAN GAUCHER FİBROBLASTLARINDA MAKROOTOFAJİ-LİZOZOMAL SİSTEMİ (MALS).
Published in:
Turkish Journal of Biochemistry / Turk Biyokimya Dergisi, 2012, v. 37, n. S1, p. 98
By:
- LAY, Incilay;
- TKACHYOVA, Ilona;
- TROPAK, Michael B.;
- RIGAT, Brigitte;
- MAHURAN, Don J.
Publication type:
Article
The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of β-glucosidase.
Published in:
FEBS Journal, 2010, v. 277, n. 7, p. 1618, doi. 10.1111/j.1742-4658.2010.07588.x
By:
- Khanna, Richie;
- Benjamin, Elfrida R.;
- Pellegrino, Lee;
- Schilling, Adriane;
- Rigat, Brigitte A.;
- Soska, Rebecca;
- Nafar, Hadis;
- Ranes, Brian E.;
- Feng, Jessie;
- Lun, Yi;
- Powe, Allan C.;
- Palling, David J.;
- Wustman, Brandon A.;
- Schiffmann, Raphael;
- Mahuran, Don J.;
- Lockhart, David J.;
- Valenzano, Kenneth J.
Publication type:
Article
NDST1 Preferred Promoter Confirmation and Identification of Corresponding Transcriptional Inhibitors as Substrate Reduction Agents for Multiple Mucopolysaccharidosis Disorders.
Published in:
PLoS ONE, 2016, v. 11, n. 9, p. 1, doi. 10.1371/journal.pone.0162145
By:
- Tkachyova, Ilona;
- Fan, Xiaolian;
- LamHonWah, Anne-Marie;
- Fedyshyn, Bohdana;
- Tein, Ingrid;
- Mahuran, Don J.;
- Schulze, Andreas
Publication type:
Article
A sensitive fluorescence-based assay for monitoring GM2 ganglioside hydrolysis in live patient cells and their lysates.
Published in:
Glycobiology, 2010, v. 20, n. 3, p. 356, doi. 10.1093/glycob/cwp183
By:
- Tropak, Michael B.;
- WBukovac, Scott;
- Rigat, Brigitte A.;
- Yonekawa, Sayuri;
- Wakarchuk, Warren;
- Mahuran, Don J.
Publication type:
Article
Identification of the hydrophobic glycoproteins of <it>Caenorhabditis elegans</it>.
Published in:
Glycobiology, 2005, v. 15, n. 10, p. 952, doi. 10.1093/glycob/cwi075
By:
- Xiaolian Fan;
- Yi-Min She;
- Richard D. Bagshaw;
- John W. Callahan;
- Harry Schachter;
- Don J. Mahuran
Publication type:
Article
Identification of Pharmacological Chaperones for Gaucher Disease and Characterization of Their Effects on β-Glucocerebrosidase by Hydrogen/Deuterium Exchange Mass Spectrometry.
Published in:
ChemBioChem, 2008, v. 9, n. 16, p. 2650, doi. 10.1002/cbic.200800304
By:
- Tropak, Michael B.;
- Kornhaber, Gregory J.;
- Rigat, Brigitte A.;
- Maegawa, Gustavo H.;
- Buttner, Justin D.;
- Blanchard, Jan E.;
- Murphy, Cecilia;
- Tuske, Steven J.;
- Coales, Stephen J.;
- Hamuro, Yoshitomo;
- Brown, Eric D.;
- Mahuran, Don J.
Publication type:
Article
Isofagomine Induced Stabilization of Glucocerebrosidase.
Published in:
ChemBioChem, 2008, v. 9, n. 16, p. 2643, doi. 10.1002/cbic.200800249
By:
- Kornhaber, Gregory J.;
- Tropak, Michael B.;
- Maegawa, Gustavo H.;
- Tuske, Steven J.;
- Coales, Stephen J.;
- Mahuran, Don J.;
- Hamuro, Yoshitomo
Publication type:
Article
Cover Picture: Identification of Pharmacological Chaperones for Gaucher Disease and Characterization of Their Effects on β-Glucocerebrosidase by Hydrogen/Deuterium Exchange Mass Spectrometry (ChemBioChem 16/2008).
Published in:
ChemBioChem, 2008, v. 9, n. 16, p. 2549, doi. 10.1002/cbic.200890061
By:
- Tropak, Michael B.;
- Kornhaber, Gregory J.;
- Rigat, Brigitte A.;
- Maegawa, Gustavo H.;
- Buttner, Justin D.;
- Blanchard, Jan E.;
- Murphy, Cecilia;
- Tuske, Steven J.;
- Coales, Stephen J.;
- Hamuro, Yoshitomo;
- Brown, Eric D.;
- Mahuran, Don J.
Publication type:
Article
W474C amino acid substitution affects early processing of the α-subunit of β-hexosaminidase A and is associated with subacute G<sub>M2</sub> gangliosidosis.
Published in:
Human Mutation, 1998, v. 11, n. 6, p. 432, doi. 10.1002/(SICI)1098-1004(1998)11:6<432::AID-HUMU3>3.0.CO;2-Z
By:
- Petroulakis, Emmanuel;
- Cao, Zhimin;
- Clarke, Joe T. R.;
- Mahuran, Don J.;
- Lee, Gregory;
- Triggs-Raine, Barbara
Publication type:
Article
A619→G substitution in the HEXB gene is not a deleterious mutation, but a frequent polymorphism.
Published in:
Human Mutation, 1998, v. 11, p. S329, doi. 10.1002/humu.13801101110
By:
- Redonnet-Vernhet, Isabelle;
- Chatelut, Martine;
- Buscail, Louis;
- Mahuran, Don J.;
- Salvayre, Robert;
- Levade, Thierry
Publication type:
Article
Lysosomal abnormalities in hereditary spastic paraplegia types SPG15 and SPG11.
Published in:
Annals of Clinical & Translational Neurology, 2014, v. 1, n. 6, p. 379, doi. 10.1002/acn3.64
By:
- Renvoisé, Benoît;
- Chang, Jaerak;
- Singh, Rajat;
- Yonekawa, Sayuri;
- FitzGibbon, Edmond J.;
- Mankodi, Ami;
- Vanderver, Adeline;
- Schindler, Alice B.;
- Toro, Camilo;
- Gahl, William A.;
- Mahuran, Don J.;
- Blackstone, Craig;
- Pierson, Tyler Mark
Publication type:
Article
Tailoring the Specificity and Reactivity of a Mechanism-Based Inactivator of Glucocerebrosidase for Potential Therapeutic Applications.
Published in:
Angewandte Chemie International Edition, 2011, v. 50, n. 44, p. 10381, doi. 10.1002/anie.201103924
By:
- Rempel, Brian P.;
- Tropak, Michael B.;
- Mahuran, Don J.;
- Withers, Stephen G.
Publication type:
Article

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