NF-κB/MAPK activation underlies ACVR1-mediated inflammation in human heterotopic ossification.

ABSTRACT:

Background

Inflammation helps regulate normal growth and tissue repair. Although bone morphogenetic proteins (BMPs) and inflammation are known contributors to abnormal bone formation, how these pathways interact in ossification remains unclear.

Methods

We examined this potential link in patients with fibrodysplasia ossificans progressiva (FOP), a genetic condition of progressive heterotopic ossification caused by activating mutations in the Activin A type I receptor (ACVR1/ALK2). FOP patients show exquisite sensitivity to trauma, suggesting that BMP pathway activation may alter immune responses. We studied primary blood, monocyte, and macrophage samples from control and FOP subjects using multiplex cytokine, gene expression, and protein analyses; examined CD14+ primary monoc

SUBMITTER: Barruet E 

PROVIDER: S-EPMC6302947 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature