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Citations to this article

Cherubism allele heterozygosity amplifies microbe-induced inflammatory responses in murine macrophages
Virginie Prod’Homme, … , Sophie Tartare-Deckert, Marcel Deckert
Virginie Prod’Homme, … , Sophie Tartare-Deckert, Marcel Deckert
Published February 23, 2015
Citation Information: J Clin Invest. 2015;125(4):1396-1400. https://doi.org/10.1172/JCI71081.
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Brief Report Immunology Article has an altmetric score of 8

Cherubism allele heterozygosity amplifies microbe-induced inflammatory responses in murine macrophages

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Abstract

Cherubism is a rare autoinflammatory bone disorder that is associated with point mutations in the SH3-domain binding protein 2 (SH3BP2) gene, which encodes the adapter protein 3BP2. Individuals with cherubism present with symmetrical fibro-osseous lesions of the jaw, which are attributed to exacerbated osteoclast activation and defective osteoblast differentiation. Although it is a dominant trait in humans, cherubism appears to be recessively transmitted in mice, suggesting the existence of additional factors in the pathogenesis of cherubism. Here, we report that macrophages from 3BP2-deficient mice exhibited dramatically reduced inflammatory responses to microbial challenge and reduced phagocytosis. 3BP2 was necessary for LPS-induced activation of signaling pathways involved in macrophage function, including SRC, VAV1, p38MAPK, IKKα/β, RAC, and actin polymerization pathways. Conversely, we demonstrated that the presence of a single Sh3bp2 cherubic allele and pathogen-associated molecular pattern (PAMP) stimulation had a strong cooperative effect on macrophage activation and inflammatory responses in mice. Together, the results from our study in murine genetic models support the notion that infection may represent a driver event in the etiology of cherubism in humans and suggest limiting inflammation in affected individuals may reduce manifestation of cherubic lesions.

Authors

Virginie Prod’Homme, Laurent Boyer, Nicholas Dubois, Aude Mallavialle, Patrick Munro, Xavier Mouska, Isabelle Coste, Robert Rottapel, Sophie Tartare-Deckert, Marcel Deckert

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Total citations by year

Year: 2025 2024 2022 2021 2020 2019 2018 2017 Total
Citations: 2 3 1 2 3 3 1 2 17
Citation information
This citation data is accumulated from CrossRef, which receives citation information from participating publishers, including this journal. Not all publishers participate in CrossRef, so this information is not comprehensive. Additionally, data may not reflect the most current citations to this article, and the data may differ from citation information available from other sources (for example, Google Scholar, Web of Science, and Scopus).

Citations to this article in year 2018 (1)

Title and authors Publication Year
Second-Generation SYK Inhibitor Entospletinib Ameliorates Fully Established Inflammation and Bone Destruction in the Cherubism Mouse Model: A SYK INHIBITOR FOR CHERUBISM TREATMENT
T Yoshimoto, T Hayashi, T Kondo, M Kittaka, EJ Reichenberger, Y Ueki
Journal of Bone and Mineral Research 2018

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