| http://purl.uniprot.org/citations/21719679 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/21719679 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/21719679 | http://www.w3.org/2000/01/rdf-schema#comment | "Mucolipidosis II is a severe lysosomal storage disorder caused by defects in the α and β subunits of the hexameric N-acetylglucosamine-1-phosphotransferase complex essential for the formation of the mannose 6-phosphate targeting signal on lysosomal enzymes. Cleavage of the membrane-bound α/β-subunit precursor by an unknown protease is required for catalytic activity. Here we found that the α/β-subunit precursor is cleaved by the site-1 protease (S1P) that activates sterol regulatory element-binding proteins in response to cholesterol deprivation. S1P-deficient cells failed to activate the α/β-subunit precursor and exhibited a mucolipidosis II-like phenotype. Thus, S1P functions in the biogenesis of lysosomes, and lipid-independent phenotypes of S1P deficiency may be caused by lysosomal dysfunction."xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.org/dc/terms/identifier | "doi:10.1126/science.1205677"xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.org/dc/terms/identifier | "doi:10.1126/science.1205677"xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/author | "Schweizer M."xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/author | "Schweizer M."xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/author | "Braulke T."xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/author | "Braulke T."xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/author | "Pohl S."xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/author | "Pohl S."xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/author | "Kollmann K."xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/author | "Kollmann K."xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/author | "Marschner K."xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/author | "Marschner K."xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/date | "2011"xsd:gYear |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/date | "2011"xsd:gYear |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/name | "Science"xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/name | "Science"xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/pages | "87-90"xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/pages | "87-90"xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/title | "A key enzyme in the biogenesis of lysosomes is a protease that regulates cholesterol metabolism."xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/title | "A key enzyme in the biogenesis of lysosomes is a protease that regulates cholesterol metabolism."xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/volume | "333"xsd:string |
| http://purl.uniprot.org/citations/21719679 | http://purl.uniprot.org/core/volume | "333"xsd:string |