Diltiazem, a L-type Ca(2+) channel blocker, also acts as a pharmacological chaperone in Gaucher patient cells

由 Rigat, Brigitte, Mahuran, Don
出版 2009

Lending a helping hand, screening chemical libraries for compounds that enhance β-hexosaminidase A activity in GM2 gangliosidosis cells

由 Tropak, Michael B., Mahuran, Don
出版 2007

Letter to the Editor

由 Mark, Brian L., Mahuran, Don
發表在 Mol Ther (2021)

A single site in human β-hexosaminidase A binds both 6-sulfate-groups on hexosamines and the sialic acid moiety of GM2 ganglioside

由 Sharma, Rohita, Bukovac, Scott, Callahan, John, Mahuran, Don
出版 2003

Role of βArg(211) in the Active Site of Human β-Hexosaminidase B

由 Hou, Yongmin, Vocadlo, David, Withers, Stephen, Mahuran, Don
出版 2000

Identification of the 6-Sulfate Binding Site Unique to α-Subunit-Containing Isozymes of Human β-Hexosaminidase

由 Sharma, Rohita, Deng, Huinan, Leung, Amy, Mahuran, Don
出版 2001

GM2 activator protein inhibits platelet activating factor signaling in rats

由 Rigat, Brigitte, Yeger, Herman, Shehnaz, Darakhshanda, Mahuran, Don
出版 2009

An α-subunit loop structure is required for GM2 activator protein binding by β-hexosaminidase A

由 Zarghooni, Maryam, Bukovac, Scott, Tropak, Michael, Callahan, John, Mahuran, Don
出版 2004

Characterization of the Biosynthesis, Processing and Kinetic Mechanism of Action of the Enzyme Deficient in Mucopolysaccharidosis IIIC

由 Fan, Xiaolian, Tkachyova, Ilona, Sinha, Ankit, Rigat, Brigitte, Mahuran, Don
出版 2011

Pharmacological chaperones facilitate the post-ER transport of recombinant N370S mutant β-glucocerebrosidase in plant cells: Evidence that N370S is a folding mutant

由 Babajani, Gholamreza, Tropak, Michael B., Mahuran, Don J., Kermode, Allison R.
出版 2012

Impaired Glucose Tolerance in a Mouse Model of Sidt2 Deficiency

由 Gao, Jialin, Gu, Xuefan, Mahuran, Don J., Wang, Zhugang, Zhang, Huiwen
出版 2013

HIGH-THROUGHPUT SCREENING FOR NOVEL HUMAN LYSOSOMAL BETA-N-ACETYL HEXOSAMINIDASE INHIBITORS ACTING AS PHARMACOLOGICAL CHAPERONES

由 Tropak, Michael B., Blanchard, Jan, Withers, Stephen G., Brown, Eric, Mahuran, Don
出版 2007

Characterization of the Glu and Asp Residues in the Active Site of Human β-Hexosaminidase B

由 Hou, Yongmin, Vocadlo, David J., Leung, Amy, Withers, Stephen G., Mahuran, Don
出版 2001

Pharmacological Enhancement of β-Hexosaminidase Activity in Fibroblasts from Adult Tay-Sachs and Sandhoff Patients

由 Tropak, Michael B., Reid, Stephen P., Guiral, Marianne, Withers, Stephen G., Mahuran, Don
出版 2004

Comparison of HCMV IE and EF-1α Promoters for the Stable Expression of β-Subunit of Hexosaminidase in CHO Cell Lines

由 Sinici, Incilay, Zarghooni, Maryam, Tropak, Michael B., Mahuran, Don J., Özkara, H. Asuman
出版 2006

Crystal Structure of β-Hexosaminidase B in Complex with Pyrimethamine, a Potential Pharmacological Chaperone()

由 Bateman, Katherine S., Cherney, Maia M., Mahuran, Don J., Tropak, Michael, James, Michael N. G.
出版 2011

A SENSITIVE FLUORESCENCE-BASED ASSAY FOR MONITORING GM2 GANGLIOSIDE HYDROLYSIS IN LIVE PATIENT CELLS AND THEIR LYSATES

由 Tropak, Michael B., Bukovac, Scott W., Rigat, Brigitte A., Yonekawa, Sayuri, Wakarchuk, Warren, Mahuran, Don J.
出版 2009

Thioglycoligase-Based Assembly of Thiodisaccharides: Screening as β-Galactosidase Inhibitors

由 Kim, Young-Wan, Chen, Hong-Ming, Kim, Jin Hyo, Müllegger, Johannes, Mahuran, Don, Withers, Stephen G.
出版 2007

Crystal Structure of Human β-Hexosaminidase B: Understanding the Molecular Basis of Sandhoff and Tay–Sachs Disease

由 Mark, Brian L., Mahuran, Don J., Cherney, Maia M., Zhao, Dalian, Knapp, Spencer, James, Michael N. G.
出版 2003

Rapid Assembly of a Library of Lipophilic Iminosugars via the Thiol–Ene Reaction Yields Promising Pharmacological Chaperones for the Treatment of Gaucher Disease

由 Goddard-Borger, Ethan D., Tropak, Michael B., Yonekawa, Sayuri, Tysoe, Christina, Mahuran, Don J., Withers, Stephen G.
出版 2012