| http://purl.uniprot.org/citations/12732614 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/12732614 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/12732614 | http://www.w3.org/2000/01/rdf-schema#comment | "Lysosome-related organelles are cell type-specific intracellular compartments with distinct morphologies and functions. The molecular mechanisms governing the formation of their unique structural features are not known. Melanosomes and their precursors are lysosome-related organelles that are characterized morphologically by intralumenal fibrous striations upon which melanins are polymerized. The integral membrane protein Pmel17 is a component of the fibrils and can nucleate their formation in the absence of other pigment cell-specific proteins. Here, we show that formation of intralumenal fibrils requires cleavage of Pmel17 by a furin-like proprotein convertase (PC). As in the generation of amyloid, proper cleavage of Pmel17 liberates a lumenal domain fragment that becomes incorporated into the fibrils; longer Pmel17 fragments generated in the absence of PC activity are unable to form organized fibrils. Our results demonstrate that PC-dependent cleavage regulates melanosome biogenesis by controlling the fibrillogenic activity of a resident protein. Like the pathologic process of amyloidogenesis, the formation of other tissue-specific organelle structures may be similarly dependent on proteolytic activation of physiological fibrillogenic substrates."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.org/dc/terms/identifier | "doi:10.1083/jcb.200302072"xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.org/dc/terms/identifier | "doi:10.1083/jcb.200302072"xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/author | "Raposo G."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/author | "Raposo G."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/author | "Berson J.F."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/author | "Berson J.F."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/author | "Marks M.S."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/author | "Marks M.S."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/author | "Tenza D."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/author | "Tenza D."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/author | "Theos A.C."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/author | "Theos A.C."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/author | "Harper D.C."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/author | "Harper D.C."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/date | "2003"xsd:gYear |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/date | "2003"xsd:gYear |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/name | "J. Cell Biol."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/name | "J. Cell Biol."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/pages | "521-533"xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/pages | "521-533"xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/title | "Proprotein convertase cleavage liberates a fibrillogenic fragment of a resident glycoprotein to initiate melanosome biogenesis."xsd:string |
| http://purl.uniprot.org/citations/12732614 | http://purl.uniprot.org/core/title | "Proprotein convertase cleavage liberates a fibrillogenic fragment of a resident glycoprotein to initiate melanosome biogenesis."xsd:string |