Fibrous dysplasia animal models: A systematic review
Abstract
BACKGROUND: Fibrous dysplasia (FD) is a rare genetic bone disorder resulting in an overproduction of cAMP leading to a structurally unsound tissue, caused by a genetic mutation in the guanine nucleotide-binding protein gene (GNAS). In order to better understand this disease, several animal models have been developed with different strategies and features.
OBJECTIVE: Conduct a systematic review to analyze and compare animal models with the causative mutation and features of FD.
METHODS: A PRISMA search was conducted in Scopus, PubMed, and Web of Science. Studies reporting an in vivo model of FD that expressed the causative mutation were included for analysis. Models without the causative mutation, but developed an FD phenotype and models of FD cell implantation were included for subanalysis.
RESULTS: Seven unique models were identified. The models were assessed and compared for their face validity, construct validity, mosaicism, and induction methods. This was based on the features of clinical FD that were reported within the categories of: macroscopic features, imaging, histology and histomorphometry, histochemical and cellular markers, and blood/urine markers.
LIMITATIONS: None of the models reported all features of FD and some features were only reported in one model. This made comparing models a challenge, but indicates areas where further research is necessary.
CONCLUSION: The benefits and disadvantages of every model were assessed from a practical and scientific…
How strong is the evidence?
Evidence Hub reads this as a systematic review, done in animal, not in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
Read the paper
Related studies
- Benign periarticular, bone and joint lipomatous lesions
- A review of the physiological and histological effects of laser osteotomy
- Quality of bone healing: perspectives and assessment techniques
- Effects of bioactive lipids and lipoproteins on bone
- Bone structure and function
- Quantifying bone structure, micro-architecture, and pathophysiology with MRI.
Go deeper in Evidence Hub
Evidence Hub adds a study data sheet (design, sample size, evidence level, key numbers), an AI summary and a chat grounded in the paper's text.