| http://purl.uniprot.org/citations/29539424 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/29539424 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/29539424 | http://www.w3.org/2000/01/rdf-schema#comment | "Cysteine cathepsins play roles during development and disease beyond their function in lysosomal protein turnover. Here, we leverage a fluorescent activity-based probe (ABP), BMV109, to track cysteine cathepsins in normal and diseased zebrafish embryos. Using this probe in a model of mucolipidosis II, we show that loss of carbohydrate-dependent lysosomal sorting alters the activity of several cathepsin proteases. The data support a pathogenic mechanism where TGF-ß signals enhance the proteolytic processing of pro-Ctsk by modulating the expression of chondroitin 4-sulfate (C4-S). In MLII, elevated C4-S corresponds with TGF-ß-mediated increases in chst11 expression. Inhibiting chst11 impairs the proteolytic activation of Ctsk and alleviates the MLII phenotypes. These findings uncover a regulatory loop between TGF-ß signaling and Ctsk activation that is altered in the context of lysosomal disease. This work highlights the power of ABPs to identify mechanisms underlying pathogenic development in living animals."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.celrep.2018.02.066"xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Bogyo M."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Bogyo M."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Azadi P."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Azadi P."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Steet R.A."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Steet R.A."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Christian C."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Christian C."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Flanagan-Steet H."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Flanagan-Steet H."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Archer-Hartmann S."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Archer-Hartmann S."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Aarnio-Peterson M."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Aarnio-Peterson M."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Lu P.N."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Lu P.N."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Sanman L."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/author | "Sanman L."xsd:string |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/date | "2018"xsd:gYear |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/date | "2018"xsd:gYear |
| http://purl.uniprot.org/citations/29539424 | http://purl.uniprot.org/core/name | "Cell Rep."xsd:string |