Inhibition of TGFβ signaling decreases osteogenic differentiation of fibrodysplasia ossificans progressiva fibroblasts in a novel in vitro model of the disease
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- 作者：Dimitra Michaa ; d.micha@vumc.nl" class="auth_mail" title="E-mail the corresponding author ; Elise Voermansa ; elisevoermans@hotmail.com" class="auth_mail" title="E-mail the corresponding author ; Marelise E.W. Eekhoffb ; EMW.Eekhoff@vumc.nl" class="auth_mail" title="E-mail the corresponding author ; Huib W. van Essenc ; HW.vanEssen@vumc.nl" class="auth_mail" title="E-mail the corresponding author ; Behrouz Zandieh-Doulabid ; bzandiehdoulabi@acta.nl" class="auth_mail" title="E-mail the corresponding author ; Coen Netelenbosb ; cnetelen@planet.nl" class="auth_mail" title="E-mail the corresponding author ; Thomas Rustemeyere ; t.rustemeyer@vumc.nl" class="auth_mail" title="E-mail the corresponding author ; E.A. Sistermansa ; e.sistermans@vumc.nl" class="auth_mail" title="E-mail the corresponding author ; Gerard Palsa ; g.pals@vumc.nl" class="auth_mail" title="E-mail the corresponding author ; Nathalie Bravenboerc ; n.bravenboer@vumc.nl" class="auth_mail" title="E-mail the corresponding author
- 关键词：FOP ; fibrodysplasia ossificans progressiva ; FCS ; fetal calf serum ; BMP ; bone morphogenetic protein ; TGFβ ; transforming growth factor β ; Runx2 ; runt-related transcription factor 2 ; Alp ; alkaline phosphatase ; OC ; osteocalcin ; ACVR1 ; activin receptor IA ; ALK2 ; activin-like kinase 2 ; HO ; heterotopic ossification ; SHED ; stem cells from human exfoliated deciduous teeth ; iPSC ; induced pluripotent stem cells ; NSAID ; nonsteroidal anti-inflammatory drug
- 刊名：Bone
- 出版年：2016
- 出版时间：March 2016
- 年：2016
- 卷：84
- 期：Complete
- 页码：169-180
- 全文大小：1817 K
文摘
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The risk of HO accompanying biopsy acquisition hinders the study of FOP.
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Osteogenic transdifferentiation of fibroblasts provides an in vitro model for FOP.
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FOP fibroblasts show higher potential for osteogenic transdifferentiation.
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TGFβ signaling inhibition stops osteogenic transdifferentiation and mineralization.
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TGFβ is an attractive new target for FOP treatment.