Excessive exosome release is the pathogenic pathway linking a lysosomal deficiency to generalized fibrosis

Lysosomal exocytosis is a ubiquitous process negatively regulated by neuraminidase 1 (NEU1), a sialidase mutated in the glycoprotein storage disease sialidosis. In Neu1(−/−) mice, excessive lysosomal exocytosis is at the basis of disease pathogenesis. Yet, the tissue-specific molecular consequences...

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Dades bibliogràfiques
Publicat a:Sci Adv
Autors principals: van de Vlekkert, Diantha, Demmers, Jeroen, Nguyen, Xinh-Xinh, Campos, Yvan, Machado, Eda, Annunziata, Ida, Hu, Huimin, Gomero, Elida, Qiu, Xiaohui, Bongiovanni, Antonella, Feghali-Bostwick, Carol A., d’Azzo, Alessandra
Format: Artigo
Idioma:Inglês
Publicat: American Association for the Advancement of Science 2019
Matèries:
Research Articles
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC6636989/
https://ncbi.nlm.nih.gov/pubmed/31328155
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1126/sciadv.aav3270
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