| http://purl.uniprot.org/citations/22128166 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/22128166 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/22128166 | http://www.w3.org/2000/01/rdf-schema#comment | "G(M1) gangliosidosis and Morquio B are autosomal recessive lysosomal storage diseases associated with a neurodegenerative disorder or dwarfism and skeletal abnormalities, respectively. These diseases are caused by deficiencies in the lysosomal enzyme β-d-galactosidase (β-Gal), which lead to accumulations of the β-Gal substrates, G(M1) ganglioside, and keratan sulfate. β-Gal is an exoglycosidase that catalyzes the hydrolysis of terminal β-linked galactose residues. This study shows the crystal structures of human β-Gal in complex with its catalytic product galactose or with its inhibitor 1-deoxygalactonojirimycin. Human β-Gal is composed of a catalytic TIM barrel domain followed by β-domain 1 and β-domain 2. To gain structural insight into the molecular defects of β-Gal in the above diseases, the disease-causing mutations were mapped onto the three-dimensional structure. Finally, the possible causes of the diseases are discussed."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.org/dc/terms/identifier | "doi:10.1074/jbc.m111.293795"xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.org/dc/terms/identifier | "doi:10.1074/jbc.m111.293795"xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/author | "Shimizu T."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/author | "Shimizu T."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/author | "Satow Y."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/author | "Satow Y."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/author | "Ochi T."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/author | "Ochi T."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/author | "Usui K."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/author | "Usui K."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/author | "Yuki K."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/author | "Yuki K."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/author | "Ohto U."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/author | "Ohto U."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/date | "2012"xsd:gYear |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/date | "2012"xsd:gYear |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/name | "J. Biol. Chem."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/name | "J. Biol. Chem."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/pages | "1801-1812"xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/pages | "1801-1812"xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/title | "Crystal structure of human beta-galactosidase: structural basis of Gm1 gangliosidosis and morquio B diseases."xsd:string |
| http://purl.uniprot.org/citations/22128166 | http://purl.uniprot.org/core/title | "Crystal structure of human beta-galactosidase: structural basis of Gm1 gangliosidosis and morquio B diseases."xsd:string |