ArticleCell reports2014
Enhanced TLR-MYD88 signaling stimulates autoinflammation in SH3BP2 cherubism mice and defines the etiology of cherubism.
Article in Cell reports, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01630447 (Identification of Mutations That Lead to Cherubism in Families and Isolated Cases and Studies of Cellular and Molecular Mechanisms), which is not on this map. Cited by 33 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Identification of Mutations That Lead to Cherubism in Families and Isolated Cases and Studies of Cellular and Molecular Mechanisms
Who cites it
33 citing papers in PubMed, 48 citations in OpenAlex.
- Exploring a cherubism bone phenotype outside the craniofacial region.Orphanet journal of rare diseases · 2026Article
- Porphyromonas gingivalis-derived extracellular vesicles aggravate bone destruction in rheumatoid arthritis by promoting Syk-dependent osteoclastogenesis.International journal of oral science · 2026Article
- Comparative analysis of osteoclast function in symptomatic and asymptomatic individuals with cherubism-causing SH3BP2 mutation.JBMR plus · 2025Article
- The bone phenotype associated with cherubism is independent of Caspase-1-dependent inflammasome activation in the mouse.PloS one · 2025Article
- Loss-of-functionJBMR plus · 2024Article
- Scaffold protein SH3BP2 signalosome is pivotal for immune activation in nephrotic syndrome.JCI insight · 2024Article
- On-Off Phagocytosis and Switchable Macrophage Activation Stimulated with NIR for Infected Percutaneous Tissue Repair of Polypyrrole-Coated Sulfonated PEEK.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2023Article
- Treatment of Progressive Cherubism during the Second Dental Transitional Phase with Calcitonin.Case reports in dentistry · 2023Article
- Overexpression of DEL-1 Downregulates SH3BP2 Expression and Inhibits Porphyromonas gingivalis-induced Gingival Inflammation In Vivo and In Vitro.Oral health & preventive dentistry · 2022Article
- Tankyrase represses autoinflammation through the attenuation of TLR2 signaling.The Journal of clinical investigation · 2022Article
- Tlr2/4-Mediated Hyperinflammation Promotes Cherubism-Like Jawbone Expansion in Sh3bp2 (P416R) Knockin Mice.JBMR plus · 2022Article
- SH3BP2 Deficiency Ameliorates Murine Systemic Lupus Erythematosus.International journal of molecular sciences · 2021Article
- Unusual Characteristics and Variable Expressivity in a Brazilian Family with Cherubism.Journal of pediatric genetics · 2021Article
- Cherubism as a systemic skeletal disease: evidence from an aggressive case.BMC musculoskeletal disorders · 2020Article
- Microbe-Dependent Exacerbated Alveolar Bone Destruction in Heterozygous Cherubism Mice.JBMR plus · 2020Article
- Article
- RANKL-independent osteoclastogenesis in the SH3BP2 cherubism mice.Bone reports · 2020Article
- Alveolar Bone Protection by Targeting the SH3BP2-SYK Axis in Osteoclasts.Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research · 2020Article
- Review
- Osteoimmunology: evolving concepts in bone-immune interactions in health and disease.Nature reviews. Immunology · 2019Review
Corrections and comments
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Authors and funding
12 authors at 4 institutions in 2 countries.
Funding
Abstract
Cherubism is caused by mutations in SH3BP2. Studies of cherubism mice showed that tumor necrosis factor α (TNF-α)-dependent autoinflammation is a major cause of the disorder but failed to explain why human cherubism lesions are restricted to jaws and regress after puberty. We demonstrate that the inflammation in cherubism mice is MYD88 dependent and is rescued in the absence of TLR2 and TLR4. However, germ-free cherubism mice also develop inflammation. Mutant macrophages are hyperresponsive to PAMPs (pathogen-associated molecular patterns) and DAMPs (damage-associated molecular patterns) that activate Toll-like receptors (TLRs), resulting in TNF-α overproduction. Phosphorylation of SH3BP2 at Y183 is critical for the TNF-α production. Finally, SYK depletion in macrophages prevents the inflammation. These data suggest that the presence of a large amount of TLR ligands, presumably oral bacteria and DAMPs during jawbone remodeling, may cause the jaw-specific development of human cherubism lesions. Reduced levels of DAMPs after stabilization of jaw remodeling may contribute to the age-dependent regression.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.