| First Author | Zhou X | Year | 2017 |
| Journal | Mol Neurodegener | Volume | 12 |
| Issue | 1 | Pages | 62 |
| PubMed ID | 28835281 | Mgi Jnum | J:269551 |
| Mgi Id | MGI:6274956 | Doi | 10.1186/s13024-017-0205-9 |
| Citation | Zhou X, et al. (2017) Lysosomal processing of progranulin. Mol Neurodegener 12(1):62 |
| abstractText | BACKGROUND: Mutations resulting in progranulin (PGRN) haploinsufficiency cause frontotemporal lobar degeneration with TDP-43-positive inclusions (FTLD-TDP), a devastating neurodegenerative disease. PGRN is localized to the lysosome and important for proper lysosome function. However, the metabolism of PGRN in the lysosome is still unclear. RESULTS: Here, we report that PGRN is processed into ~10 kDa peptides intracellularly in multiple cell types and tissues and this processing is dependent on lysosomal activities. PGRN endocytosed from the extracellular space is also processed in a similar manner. We further demonstrated that multiple cathepsins are involved in PGRN processing and cathepsin L cleaves PGRN in vitro. CONCLUSIONS: Our data support that PGRN is processed in the lysosome through the actions of cathepsins. |
