1now Summary

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

The structure was published by Mark, B.L., Mahuran, D.J., Cherney, M.M., Zhao, D., Knapp, S., and James, M.N.G., in 2003 in a paper entitled "Crystal structure of Human beta-hexosaminidase B: Understanding the molecular basis of Sandhoff and Tay-Sachs disease" (abstract).

This crystal structure was determined using X-ray diffraction at a resolution of 2.2 Å and deposited in 2003.

The experimental data on which the structure is based was also deposited.

This PDB entry contains multiple copies of the structure of beta-hexosaminidase beta chain.

It also contains one or more heterogenic compounds (e.g., ligands, co-factors, ions, modified amino acids, etc.); see here for a complete list.

The molecule has more than one probable quaternary state observed. For more details see the quaternary structure page.

The following tables show cross-reference information to other databases (to obtain a list of all PDB entries sharing the same property or classification, click on the magnifying glass icon):


Chain Name UniProt Name of source organism % of UniProt sequence present in the sample Residues in the sample molecules % of residues observed
A beta-hexosaminidase beta chain P07686 (50-556) (HEXB_HUMAN)search Homo sapienssearch 98% 507 94%
B beta-hexosaminidase beta chain P07686 (50-556) (HEXB_HUMAN)search Homo sapienssearch 98% 507 94%


This entry contains 1 unique UniProt protein:

UniProt accession Name Organism PDB
P07686 (50 - 556) beta-hexosaminidase beta chain Homo sapiens

Chain Structural classification (SCOP) Structural classification (CATH) Sequence family (Pfam)
A, B (P07686) beta-N-acetylhexosaminidase catalytic domainsearch, beta-N-acetylhexosaminidase domainsearch Chitobiase, domain 2search, Glycosidasessearch PF00728: Glycosyl hydrolase family 20, catalytic domainsearch, PF14845: beta-acetyl hexosaminidase likesearch

Chain ID Biological process (GO) Cellular component (GO) Molecular function (GO)
A, B (P07686) glycosaminoglycan metabolic processsearch neuromuscular process controlling balancesearch lipid storagesearch astrocyte cell migrationsearch cell deathsearch phospholipid biosynthetic processsearch oligosaccharide catabolic processsearch hyaluronan catabolic processsearch carbohydrate metabolic processsearch cellular protein metabolic processsearch penetration of zona pellucidasearch hyaluronan metabolic processsearch ganglioside catabolic processsearch chondroitin sulfate metabolic processsearch male courtship behaviorsearch regulation of cell shapesearch sensory perception of soundsearch glycosphingolipid metabolic processsearch positive regulation of transcription from RNA polymerase II promotersearch metabolic processsearch small molecule metabolic processsearch cellular calcium ion homeostasissearch myelinationsearch single fertilizationsearch neuromuscular processsearch chondroitin sulfate catabolic processsearch keratan sulfate metabolic processsearch keratan sulfate catabolic processsearch sexual reproductionsearch skeletal system developmentsearch locomotory behaviorsearch oogenesissearch lysosome organizationsearch regulation of cellular metabolic processsearch sphingolipid metabolic processsearch lysosomal lumensearch extracellular vesicular exosomesearch lysosomesearch membranesearch acrosomal vesiclesearch protein homodimerization activitysearch beta-N-acetylhexosaminidase activitysearch protein heterodimerization activitysearch hydrolase activity, acting on glycosyl bondssearch hydrolase activity, hydrolyzing O-glycosyl compoundssearch hydrolase activitysearch

Chain InterPro annotation
A, B Glycoside hydrolase, catalytic domainsearch Glycoside hydrolase family 20, catalytic coresearch Glycoside hydrolase superfamilysearch Beta-hexosaminidasesearch Chitobiase/beta-hexosaminidase domain 2-likesearch Beta-hexosaminidase, eukaryotic type, N-terminalsearch