Ontology highlight
ABSTRACT:
SUBMITTER: Flanagan-Steet H
PROVIDER: S-EPMC6247414 | biostudies-other | 2018 Mar
REPOSITORIES: biostudies-other
Flanagan-Steet Heather H Christian Courtney C Lu Po-Nien PN Aarnio-Peterson Megan M Sanman Laura L Archer-Hartmann Stephanie S Azadi Parastoo P Bogyo Matthew M Steet Richard A RA
Cell reports 20180301 11
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 ...[more]