2V3D

acid-beta-glucosidase with N-butyl-deoxynojirimycin

Summary for 2V3D

Entry DOI	10.2210/pdb2v3d/pdb
Related	1OGS 1Y7V 2F61 2J25 2V3E 2V3F
Descriptor	GLUCOSYLCERAMIDASE, beta-D-mannopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-[alpha-L-fucopyranose-(1-3)]2-acetamido-2-deoxy-beta-D-glucopyranose, SULFATE ION, ... (6 entities in total)
Functional Keywords	acid-beta-glucosidase, n-butyl-deoxynojirimycinalternative initiation, sphingolipid metabolism, gaucher disease, disease mutation, lipid metabolism, polymorphism, glycoprotein, pharmaceutical, n-butyl-deoxynojirimycin, alternative splicing, membrane, lysosome, hydrolase, glycosidase
Biological source	HOMO SAPIENS (HUMAN)
Total number of polymer chains	2
Total formula weight	116072.20
Authors	Brumshtein, B.,Greenblatt, H.M.,Butters, T.D.,Shaaltiel, Y.,Aviezer, D.,Silman, I.,Futerman, A.H.,Sussman, J.L. (deposition date: 2007-06-17, release date: 2007-08-14, Last modification date: 2023-12-13)
Primary citation	Brumshtein, B.,Greenblatt, H.M.,Butters, T.D.,Shaaltiel, Y.,Aviezer, D.,Silman, I.,Futerman, A.H.,Sussman, J.L. Crystal Structures of Complexes of N-Butyl- and N-Nonyl-Deoxynojirimycin Bound to Acid Beta-Glucosidase: Insights Into the Mechanism of Chemical Chaperone Action in Gaucher Disease. J.Biol.Chem., 282:29052-, 2007 Cited by PubMed: 17666401 DOI: 10.1074/JBC.M705005200 PDB entries with the same primary citation
Experimental method	X-RAY DIFFRACTION (1.96 Å)

Structure validation