CAS 75172-81-5 Deoxygalactonojirimycin hydrochloride - Analytical Products / Alfa Chemistry

CAT	AP75172815
CAS	75172-81-5
Structure
MDL Number	MFCD00269962
Molecular Weight	199.63
InChI Key	ZJIHMALTJRDNQI-OLALXQGDSA-N

Size	2MG
Storage Conditions	−20°C

1

A Pharmacogenetic Approach to Identify Mutant Forms of α-galactosidase A That Respond to a Pharmacological Chaperone for Fabry Disease

Xiaoyang Wu, Evan Katz, Maria Cecilia Della Valle, Kirsten Mascioli, John J Flanagan, Jeffrey P Castelli, Raphael Schiffmann, Pol Boudes, David J Lockhart, Kenneth J Valenzano, Elfrida R Benjamin

Hum Mutat. 2011 Aug;32(8):965-77.

PMID: 21598360

1

Co-administration With the Pharmacological Chaperone AT1001 Increases Recombinant Human α-galactosidase A Tissue Uptake and Improves Substrate Reduction in Fabry Mice

Elfrida R Benjamin, Richie Khanna, Adriane Schilling, John J Flanagan, Lee J Pellegrino, Nastry Brignol, Yi Lun, Darlene Guillen, Brian E Ranes, Michelle Frascella, Rebecca Soska, Jessie Feng, Leo Dungan, etc.

Mol Ther. 2012 Apr;20(4):717-26.

PMID: 22215019

1

Fabry Disease: Polymorphic Haplotypes and a Novel Missense Mutation in the GLA Gene

L Ferri, C Guido, G la Marca, S Malvagia, C Cavicchi, A Fiumara, R Barone, R Parini, D Antuzzi, C Feliciani, A Zampetti, R Manna, S Giglio, C M Della Valle, X Wu, K J Valenzano, R Benjamin, M A Donati, etc.

Clin Genet. 2012 Mar;81(3):224-33.

PMID: 21517827

1

The Pharmacological Chaperone 1-deoxygalactonojirimycin Increases Alpha-Galactosidase A Levels in Fabry Patient Cell Lines

E R Benjamin, J J Flanagan, A Schilling, H H Chang, L Agarwal, E Katz, X Wu, C Pine, B Wustman, R J Desnick, D J Lockhart, K J Valenzano

J Inherit Metab Dis. 2009 Jun;32(3):424-40.

PMID: 19387866

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