Fibrodysplasia Ossificans Progressiva (FOP)
Animal models have played a critical role in advancing our understanding of fibrodysplasia ossificans progressiva (FOP) pathogenesis and in the development of potential therapies. At our company, we are committed to offering professional and reliable disease modeling services for FOP and other rare diseases. Our disease modeling services include the generation and characterization of genetically modified animal models, including mouse models, zebrafish models, and other model organisms. These models are valuable for high-throughput drug screening to identify potential therapeutic compounds.
Background of Fibrodysplasia Ossificans Progressiva (FOP)
FOP, also known as myositis ossificans progressiva (MOP), is a rare genetic disorder characterized by the progressive ossification of soft tissue, leading to bone formation in muscles, tendons, and ligaments. This disease is caused by mutations in the ACVR1 gene, which encodes a protein involved in the regulation of bone morphogenetic protein (BMP) signaling.
The typical FOP has two features: malformation of the big toes and progressive heterotopic ossification (HO) formation. According to statistics, the incidence rate of FOP is about 1/2 million, without racial, gender, and regional differences. At present, no effective cure has been found for this debilitating disease. Therefore, animal models of FOP are needed to study the mechanisms of progression of the disease.
Fig. 1 A genetically accurate and physiologically relevant mouse model of FOP. (Wolken D M A, et al., 2018)Our Services
We help our clients generate various FOP animal models using advanced technologies, including chemical mutagenesis, transgenesis, and CRISPR/Cas9 editing. Each animal model has its own advantages and limitations. For example, the zebrafish model allows for live cell lineage tracing and high-throughput drug screening due to its small size and transparency. We develop and select appropriate animal models based on the specific research needs of our clients. We also offer phenotypic characterization services of animal models, including histological analysis, imaging, and molecular analysis.
Our animal modeling services include:
- Generation of mouse models
ACVR1 is important for many aspects of mouse embryonic development and loss or over-activation of ACVR1 can cause embryonic or perinatal lethality. Therefore, our work on the FOP adult mouse model focuses on the use of chimeric/mosaic animals, or the use of various conditional gene expression methods, most commonly Cre-Lox recombination. Our mouse models include ACVR1Q207D and ACVR1R206H models.
We also provide our clients with non-ACVR1 mediated mouse models of HO development, such as transgenic BMP4 overexpression and Matrigel BMP2 delivery to identify progenitor cell populations that contribute to the development of heterotopic ossification. - Generation of embryonic chicken model
We help our clients overexpress ACVR1R206H, ACVR1Q207D, and ACVR1Q207E in chicken limbs to mimic FOP-like phenotypes, such as joint fusion and ectopic cartilage formation. - Generation of zebrafish models
We help our clients generate transgenic zebrafish expressing heat-shock-inducible Acvr1lQ204D, which exhibit some FOP-like characteristics, including HO, vertebral fusion, spinal lordosis, and fin malformations.
As a leading service provider in the field of disease modeling, we have access to the latest technologies in genome editing, imaging, and drug screening, allowing us to provide the most comprehensive disease modeling services for fibrodysplasia ossificans progressiva (FOP) disease. We have a strong commitment to ethical and responsible animal research. If you are interested in our disease modeling services, please contact us for more information.
Reference
- Wolken, D. M. A.; et al. The obligatory role of Activin A in the formation of heterotopic bone in Fibrodysplasia Ossificans Progressiva. Bone, 2018, 109: 210-217.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.
