7P2Z

Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with cyclosulfamidate 4

This is a non-PDB format compatible entry.

Summary for 7P2Z

• Entry DOI: 10.2210/pdb7p2z/pdb
• Descriptor: Lysosomal alpha-glucosidase, (3~{a}~{R},4~{S},5~{S},6~{S},7~{R},7~{a}~{S})-7-(hydroxymethyl)-2,2-bis(oxidanylidene)-3~{a},4,5,6,7,7~{a}-hexahydro-3~{H}-benzo[d][1,2,3]oxathiazole-4,5,6-triol, TRIETHYLENE GLYCOL, ... (12 entities in total)
• Functional Keywords: alpha-glycosidase, lysosomal, pompe disease, pharmacological chaperone, hydrolase
• Biological source: Homo sapiens (Human)
• Total number of polymer chains: 2
• Total formula weight: 198272.11

Authors

Roig-Zamboni, V.,Kok, K.,Overkleeft, H.,Artola, M.,Sulzenbacher, G. (deposition date: 2021-07-06, release date: 2022-07-13, Last modification date: 2024-01-31)

• Primary citation: Kok, K.,Kuo, C.L.,Katzy, R.E.,Lelieveld, L.T.,Wu, L.,Roig-Zamboni, V.,van der Marel, G.A.,Codee, J.D.C.,Sulzenbacher, G.,Davies, G.J.,Overkleeft, H.S.,Aerts, J.M.F.G.,Artola, M.; 1,6- epi-Cyclophellitol Cyclosulfamidate Is a Bona Fide Lysosomal alpha-Glucosidase Stabilizer for the Treatment of Pompe Disease.; J.Am.Chem.Soc., 144:14819-14827, 2022; PubMed: 35917590; DOI: 10.1021/jacs.2c05666

Experimental method

X-RAY DIFFRACTION (1.85 Å)