Activin A amplifies dysregulated BMP signaling and induces chondro-osseous differentiation of primary connective tissue progenitor cells in patients with fibrodysplasia ossificans progressiva (FOP)

Bone ◽  
2018 ◽  
Vol 109 ◽  
pp. 218-224 ◽  
Author(s):  
Haitao Wang ◽  
Eileen M. Shore ◽  
Robert J. Pignolo ◽  
Frederick S. Kaplan
Keyword(s):  
Connective Tissue ◽  
Progenitor Cells ◽  
Activin A ◽  
Bmp Signaling ◽  
Fibrodysplasia Ossificans Progressiva ◽  
Connective Tissue Progenitor Cells

ECSIT links TLR and BMP signaling in FOP connective tissue progenitor cells

Bone ◽  
2018 ◽  
Vol 109 ◽  
pp. 201-209 ◽  
Author(s):  
Haitao Wang ◽  
Edward M. Behrens ◽  
Robert J. Pignolo ◽  
Frederick S. Kaplan
Keyword(s):  
Connective Tissue ◽  
Progenitor Cells ◽  
Bmp Signaling ◽  
Connective Tissue Progenitor Cells

scholarly journals βig-h3 Potentiates the Profibrogenic Effect of TGFβ Signaling on Connective Tissue Progenitor Cells Through the Negative Regulation of Master Chondrogenic Genes

2013 ◽  
Vol 19 (3-4) ◽  
pp. 448-457 ◽  
Author(s):  
Carlos I. Lorda-Diez ◽  
Juan A. Montero ◽  
Manuel J. Diaz-Mendoza ◽  
Juan A. Garcia-Porrero ◽  
Juan M. Hurle
Keyword(s):  
Connective Tissue ◽  
Progenitor Cells ◽  
Negative Regulation ◽  
Tgfβ Signaling ◽  
Connective Tissue Progenitor Cells

scholarly journals The role of Activin A in fibrodysplasia ossificans progressiva: a prominent mediator

2019 ◽  
Vol 39 (8) ◽  
Author(s):  
Hui Lin ◽  
Fuli Shi ◽  
Jiayu Gao ◽  
Ping Hua
Keyword(s):  
Signaling Pathway ◽  
Genetic Disorder ◽  
Activin A ◽  
Bmp Signaling ◽  
The Body ◽  
Fibrodysplasia Ossificans Progressiva ◽  
Type I ◽  
Heterotopic Bone ◽  
Heterotopic Bone Formation

Abstract Heterotopic ossification (HO) is the aberrant formation of mature, lamellar bone in nonosseous tissue. Fibrodysplasia ossificans progressiva (FOP) is a rare and devastating genetic disorder that causes progressive HO in the ligaments, tendons, and muscles throughout the body. FOP is attributed to an autosomal mutation in activin receptor-like kinase 2 (ALK2), a bone morphogenetic protein (BMP) type I receptor. Initial studies show that mutant ALK2 drives HO by constitutively activating the BMP signaling pathway. Recently, mutant ALK2 has been shown to transduce Smad1/5 signaling and enhance chondrogenesis, calcification in response to Activin A, which normally signals through Smad2/3 and inhibits BMP signaling pathway. Furthermore, Activin A induces heterotopic bone formation via mutant ALK2, while inhibition of Activin A blocks spontaneous and trauma-induced HO. In this manuscript, we describe the molecular mechanism of the causative gene ALK2 in FOP, mainly focusing on the prominent role of Activin A in HO. It reveals a potential strategy for prevention and treatment of FOP by inhibition of Activin A. Further studies are needed to explore the cellular and molecular mechanisms of Activin A in FOP in more detail.

scholarly journals The effect of oxygen tension on the in vitro assay of human osteoblastic connective tissue progenitor cells

2008 ◽  
Vol 26 (10) ◽  
pp. 1390-1397 ◽  
Author(s):  
Sandra M. Villarruel ◽  
Cynthia A. Boehm ◽  
Mark Pennington ◽  
Jason A. Bryan ◽  
Kimerly A. Powell ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Progenitor Cells ◽  
Oxygen Tension ◽  
In Vitro Assay ◽  
Vitro Assay ◽  
Effect Of Oxygen ◽  
Connective Tissue Progenitor Cells

Growth of connective tissue progenitor cells on microtextured polydimethylsiloxane surfaces

2002 ◽  
Vol 62 (4) ◽  
pp. 499-506 ◽  
Author(s):  
Alvaro Mata ◽  
Cynthia Boehm ◽  
Aaron J. Fleischman ◽  
George Muschler ◽  
Shuvo Roy
Keyword(s):  
Connective Tissue ◽  
Progenitor Cells ◽  
Connective Tissue Progenitor Cells

scholarly journals Trochanteric Bursa Is a Source of Connective Tissue Progenitor Cells

2021 ◽  
Author(s):  
Matthew R. LeVasseur ◽  
Benjamin C. Hawthorne ◽  
Michael R. Mancini ◽  
Mary Beth R. McCarthy ◽  
Ian J. Wellington ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Progenitor Cells ◽  
Connective Tissue Progenitor Cells

scholarly journals Activin A-Smad Signaling Mediates Connective Tissue Growth Factor Synthesis in Liver Progenitor Cells

2016 ◽  
Vol 17 (3) ◽  
pp. 408 ◽  
Author(s):  
Ze-Yang Ding ◽  
Guan-Nan Jin ◽  
Wei Wang ◽  
Yi-Min Sun ◽  
Wei-Xun Chen ◽  
...  
Keyword(s):  
Growth Factor ◽  
Connective Tissue ◽  
Progenitor Cells ◽  
Connective Tissue Growth Factor ◽  
Activin A ◽  
Tissue Growth ◽  
Smad Signaling
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