1NOW

Human lysosomal beta-hexosaminidase isoform B in complex with (2R,3R,4S,5R)-2-Acetamido-3,4-Dihydroxy-5-Hydroxymethyl-Piperidinium Chloride (GalNAc-isofagomine)

Summary for 1NOW

Entry DOI	10.2210/pdb1now/pdb
Related	1NOU
Descriptor	beta-hexosaminidase beta chain, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, (2R,3R,4S,5R)-2-ACETAMIDO-3,4-DIHYDROXY-5-HYDROXYMETHYL-PIPERIDINE, ... (6 entities in total)
Functional Keywords	(beta/alpha)8-barrel, homodimer, family 20 glycosidase, hydrolase
Biological source	Homo sapiens (human)
Total number of polymer chains	2
Total formula weight	117999.35
Authors	Mark, B.L.,Mahuran, D.J.,Cherney, M.M.,Zhao, D.,Knapp, S.,James, M.N.G. (deposition date: 2003-01-16, release date: 2003-04-29, Last modification date: 2020-07-29)
Primary citation	Mark, B.L.,Mahuran, D.J.,Cherney, M.M.,Zhao, D.,Knapp, S.,James, M.N.G. Crystal structure of Human beta-hexosaminidase B: Understanding the molecular basis of Sandhoff and Tay-Sachs disease J.Mol.Biol., 327:1093-1109, 2003 Cited by PubMed: 12662933 DOI: 10.1016/S0022-2836(03)00216-X PDB entries with the same primary citation
Experimental method	X-RAY DIFFRACTION (2.2 Å)

Structure validation