A negative feedback loop between fibroadipogenic progenitors and muscle fibres involving endothelin promotes human muscle fibrosis.

ABSTRACT:

Background

Fibrosis is defined as an excessive accumulation of extracellular matrix (ECM) components. Many organs are subjected to fibrosis including the lung, liver, heart, skin, kidney, and muscle. Muscle fibrosis occurs in response to trauma, aging, or dystrophies and impairs muscle function. Fibrosis represents a hurdle for the treatment of human muscular dystrophies. While data on the mechanisms of fibrosis have mostly been investigated in mice, dystrophic mouse models often do not recapitulate fibrosis as observed in human patients. Consequently, the cellular and molecular mechanisms that lead to fibrosis in human muscle still need to be identified.

Methods

Combining mass cytometry, transcriptome profiling, in vitro co-culture experiments, and in vivo transplantation i

SUBMITTER: Bensalah M 

PROVIDER: S-EPMC9178170 | biostudies-literature | 2022 Jun

REPOSITORIES: biostudies-literature