4NXS

Crystal structure of human alpha-galactosidase A in complex with 1-deoxygalactonojirimycin-pFPhT

Summary for 4NXS

Entry DOI	10.2210/pdb4nxs/pdb
Descriptor	Alpha-galactosidase A, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, SULFATE ION, ... (6 entities in total)
Functional Keywords	pharmacological chaperone, drug delivery systems, enzyme activation, enzyme stability, humans, lysosomes, molecular chaperones, protein binding, alpha-galactosidase, hydrolase-hydrolase inhibitor complex, fabry disease, gla gene, arylthiourea, hydrolase/hydrolase inhibitor
Biological source	Homo sapiens (human)
Total number of polymer chains	2
Total formula weight	93635.73
Authors	Johnson, J.L.,Drury, J.E.,Lieberman, R.L. (deposition date: 2013-12-09, release date: 2014-06-11, Last modification date: 2023-09-20)
Primary citation	Yu, Y.,Mena-Barragan, T.,Higaki, K.,Johnson, J.L.,Drury, J.E.,Lieberman, R.L.,Nakasone, N.,Ninomiya, H.,Tsukimura, T.,Sakuraba, H.,Suzuki, Y.,Nanba, E.,Mellet, C.O.,Garcia Fernandez, J.M.,Ohno, K. Molecular Basis of 1-Deoxygalactonojirimycin Arylthiourea Binding to Human alpha-Galactosidase A: Pharmacological Chaperoning Efficacy on Fabry Disease Mutants. Acs Chem.Biol., 9:1460-1469, 2014 Cited by PubMed: 24783948 DOI: 10.1021/cb500143h PDB entries with the same primary citation
Experimental method	X-RAY DIFFRACTION (2.5493 Å)

Structure validation