ESPEYB20 3. Bone, Growth Plate and Mineral Metabolism Translational Highlights (2 abstracts)
3.12. Impaired bone strength and bone microstructure in a novel early-onset osteoporotic rat model with a clinically relevant PLS3 mutation
Hu J , Zhou B , Lin X , Zhang Q , Guan F , Sun L , Liu J , Wang O , Jiang Y , Xia WB , Xing X & Li M
Elife. 2023 Apr 21;12:e80365. doi: 10.7554/eLife.80365. PMID: 37083757. https://pubmed.ncbi.nlm.nih.gov/37083757/
In Brief: The study established a novel rat model with a clinically relevant PLS3 mutation, which replicates the osteoporotic phenotype of early-onset PLS3-related osteoporosis. The findings suggest that treatment with alendronate or teriparatide improves bone mass and microarchitecture, suggesting their potential as effective treatments for early-onset osteoporosis caused by PLS3 mutations.
Commentary: This study is an essential step forward in understanding early-onset osteoporosis caused by mutations in PLS3, which encodes a protein crucial to bone health. The creation of a rat model with a clinically relevant PLS3 mutation is important as it effectively mimics the osteoporotic conditions seen in humans. This model further provided insights into the role of PLS3 in bone microstructure and strength regulation, highlighting its critical role in maintaining bone health. By observing the decreased bone strength and thin cortical thickness in the rat model, researchers were able to gain a clearer understanding of the impacts of PLS3 mutations on human bone health.
Importantly, the finding that alendronate and teriparatide dramatically improve bone mass and microarchitecture in this rat model offers hope for therapeutic advances in treatment of PLS3-induced osteoporosis. Alendronate and teriparatide have long been used in the treatment of osteoporosis, but their efficacy in the context of a PLS3 mutation has not been established. This new evidence underscores their potential in treating early-onset osteoporosis. Ultimately, these findings add to our understanding the role of PLS3 in bone health, and also open new avenues to develop and test novel treatment strategies for early-onset osteoporosis.
Volume 20
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ESPE Yearbook of Paediatric Endocrinology
ISSN 1662-4009 (Online)
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