n/a
Article Publish Status: FREE
Abstract Title:

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

Abstract Source:

Sci Adv. 2019 07 ;5(7):eaav3270. Epub 2019 Jul 17. PMID: 31328155

Abstract Author(s):

Diantha van de Vlekkert, Jeroen Demmers, Xinh-Xinh Nguyen, Yvan Campos, Eda Machado, Ida Annunziata, Huimin Hu, Elida Gomero, Xiaohui Qiu, Antonella Bongiovanni, Carol A Feghali-Bostwick, Alessandra d'Azzo

Article Affiliation:

Diantha van de Vlekkert

Abstract:

Lysosomal exocytosis is a ubiquitous process negatively regulated by neuraminidase 1 (NEU1), a sialidase mutated in the glycoprotein storage disease sialidosis. Inmice, excessive lysosomal exocytosis is at the basis of disease pathogenesis. Yet, the tissue-specific molecular consequences of this deregulated pathway are still unfolding. We now report that in muscle connective tissue,fibroblasts have features of myofibroblasts and are proliferative, migratory, and exocytose large amounts of exosomes. These nanocarriers loaded with activated transforming growth factor-β and wingless-related integration site (WNT)/β-catenin signaling molecules propagate fibrotic signals to other cells, maintaining the tissue in a prolonged transitional status. Myofibroblast-derived exosomes fed to normal fibroblasts convert them into myofibroblasts, changing the recipient cells'proliferative and migratory properties. These findings reveal an unexpected exosome-mediated signaling pathway downstream of NEU1 deficiency that propagates a fibrotic disease and could be implicated in idiopathic forms of fibrosis in humans.

Study Type : In Vitro Study
Additional Links
Additional Keywords : Exosomes : CK(312) : AC(199)

Print Options


Key Research Topics

Sayer Ji
Founder of GreenMedInfo.com

Subscribe to our informative Newsletter & get Nature's Evidence-Based Pharmacy

Our newsletter serves 500,000 with essential news, research & healthy tips, daily.

Download Now

500+ pages of Natural Medicine Alternatives and Information.

This website is for information purposes only. By providing the information contained herein we are not diagnosing, treating, curing, mitigating, or preventing any type of disease or medical condition. Before beginning any type of natural, integrative or conventional treatment regimen, it is advisable to seek the advice of a licensed healthcare professional.

© Copyright 2008-2021 GreenMedInfo.com, Journal Articles copyright of original owners, MeSH copyright NLM.