| http://purl.uniprot.org/citations/33980489 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/33980489 | http://www.w3.org/2000/01/rdf-schema#comment | "The enzymes β-galactosidase (GLB1) and neuraminidase 1 (NEU1; sialidase 1) participate in the degradation of glycoproteins and glycolipids in the lysosome. To remain active and stable, they associate with PPCA [protective protein cathepsin A (CTSA)] into a high-molecular weight lysosomal multienzyme complex (LMC), of which several forms exist. Genetic defects in these three proteins cause the lysosomal storage diseases GM1-gangliosidosis/mucopolysaccharidosis IV type B, sialidosis, and galactosialidosis, respectively. To better understand the interactions between these enzymes, we determined the three-dimensional structure of the murine LMC core. This 0.8-MDa complex is composed of three GLB1 dimers and three CTSA dimers, adopting a triangular architecture maintained through six copies of a unique GLB1-CTSA polar interface. Mutations in this contact surface that occur in GM1-gangliosidosis prevent formation of the LMC in vitro. These findings may facilitate development of therapies for lysosomal storage disorders."xsd:string |
| http://purl.uniprot.org/citations/33980489 | http://purl.org/dc/terms/identifier | "doi:10.1126/sciadv.abf4155"xsd:string |
| http://purl.uniprot.org/citations/33980489 | http://purl.uniprot.org/core/author | "Nagar B."xsd:string |
| http://purl.uniprot.org/citations/33980489 | http://purl.uniprot.org/core/author | "Gorelik A."xsd:string |
| http://purl.uniprot.org/citations/33980489 | http://purl.uniprot.org/core/author | "Illes K."xsd:string |
| http://purl.uniprot.org/citations/33980489 | http://purl.uniprot.org/core/author | "Mazhab-Jafari M.T."xsd:string |
| http://purl.uniprot.org/citations/33980489 | http://purl.uniprot.org/core/author | "Hasan S.M.N."xsd:string |
| http://purl.uniprot.org/citations/33980489 | http://purl.uniprot.org/core/date | "2021"xsd:gYear |
| http://purl.uniprot.org/citations/33980489 | http://purl.uniprot.org/core/name | "Sci Adv"xsd:string |
| http://purl.uniprot.org/citations/33980489 | http://purl.uniprot.org/core/pages | "eabf4155"xsd:string |
| http://purl.uniprot.org/citations/33980489 | http://purl.uniprot.org/core/title | "Structure of the murine lysosomal multienzyme complex core."xsd:string |
| http://purl.uniprot.org/citations/33980489 | http://purl.uniprot.org/core/volume | "7"xsd:string |
| http://purl.uniprot.org/citations/33980489 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/33980489 |
| http://purl.uniprot.org/citations/33980489 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/33980489 |
| http://purl.uniprot.org/uniprot/#_P23780-mappedCitation-33980489 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/33980489 |
| http://purl.uniprot.org/uniprot/#_P16675-mappedCitation-33980489 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/33980489 |
| http://purl.uniprot.org/uniprot/P23780 | http://purl.uniprot.org/core/mappedCitation | http://purl.uniprot.org/citations/33980489 |
| http://purl.uniprot.org/uniprot/P16675 | http://purl.uniprot.org/core/mappedCitation | http://purl.uniprot.org/citations/33980489 |