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    Osteochondroma Pathogenesis: Mouse Models and Mechanistic Insights into Interactions with Retinoid Signaling

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    Author
    Garcia, Sonia Arely
    Ng, Vincent Y
    Iwamoto, Masahiro
    Enomoto-Iwamoto, Motomi
    Date
    2021-11-19
    Journal
    American Journal of Pathology
    Publisher
    Elsevier Inc.
    Type
    Article
    
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    Show full item record
    See at
    https://doi.org/10.1016/j.ajpath.2021.08.003
    Abstract
    Osteochondromas are cartilage-capped tumors that arise near growing physes and are the most common benign bone tumor in children. Osteochondromas can lead to skeletal deformity, pain, loss of motion, and neurovascular compression. Currently, surgery is the only available treatment for symptomatic osteochondromas. Osteochondroma mouse models have been developed to understand the pathology and the origin of osteochondromas and develop therapeutic drugs. Several cartilage regulatory pathways have been implicated in the development of osteochondromas, such as bone morphogenetic protein, hedgehog, and WNT/β-catenin signaling. Retinoic acid receptor-γ is an important regulator of endochondral bone formation. Selective agonists for retinoic acid receptor-γ, such as palovarotene, have been investigated as drugs for inhibition of ectopic endochondral ossification, including osteochondromas. This review discusses the signaling pathways involved in osteochondroma pathogenesis and their possible interactions with the retinoid pathway.
    Rights/Terms
    Copyright © 2021 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.
    Keyword
    Cartilage tumor
    Endochondral ossification
    Growth plate
    Osteochondroma
    Retinoids
    Identifier to cite or link to this item
    http://hdl.handle.net/10713/17197
    ae974a485f413a2113503eed53cd6c53
    10.1016/j.ajpath.2021.08.003
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