Recapitulation of pro-inflammatory signature of monocytes with ACVR1A mutation using FOP patient-derived iPSCs

Recapitulation of pro-inflammatory signature of monocytes with ACVR1A mutation using FOP patient-derived iPSCs

Abstract Background Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disease characterized by progressive heterotopic ossification (HO) in soft tissues due to a heterozygous mutation of the ACVR1A gene (FOP-ACVR1A), which erroneously transduces the BMP signal by Activin-A. Although infl...

Full description

Bibliographic Details
Main Authors: Hirotsugu Maekawa, Yonghui Jin, Megumi Nishio, Shunsuke Kawai, Sanae Nagata, Takeshi Kamakura, Hiroyuki Yoshitomi, Akira Niwa, Megumu K. Saito, Shuichi Matsuda, Junya Toguchida
Format: Article
Language:English
Published: BMC 2022-09-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Fibrodysplasia ossificans progressive (FOP)
Monocyte
Inflammation
Induced pluripotent stem cell (iPSC)
Activin-A
Bone morphogenic protein (BMP)
Online Access:https://doi.org/10.1186/s13023-022-02506-3

Internet

https://doi.org/10.1186/s13023-022-02506-3

Similar Items