Osteogenesis Imperfecta (OI) Animal Model Service
Osteogenesis Imperfecta (OI) mouse models provide critical insights into bone pathophysiology and serve as indispensable platforms for evaluating novel therapeutics. At Protheragen, we specialize in developing highly precise animal models to accelerate preclinical research for potential OI therapies. Our expertise ensures that clients receive the most reliable and physiologically relevant models, ultimately streamlining their drug development pipeline.
Overview of Osteogenesis Imperfecta (OI) Animal Models
Animal models of osteogenesis imperfecta (OI) primarily consist of genetically engineered mice that recapitulate various mutations in human collagen-related genes. These models consistently display core hallmarks of the disease, including reduced bone density, increased bone fragility, skeletal deformities, and altered bone matrix composition. They are indispensable tools for elucidating disease mechanisms, studying bone biomechanics, and evaluating various therapeutic interventions, ranging from bisphosphonates and anabolic drugs to novel gene and cell therapies.
Fig.1 Bone properties were assessed in the osteogenesis imperfecta (OI) mouse model. (Alcorta-Sevillano N, et al., 2022)
Our Services
Protheragen empowers researchers in rare musculoskeletal diseases with highly accurate, customizable animal models. Our osteogenesis imperfecta (OI) models are rigorously validated to faithfully recapitulate human pathology, bridging the gap between basic research and clinical application. We provide the critical preclinical tools you need to reliably study mechanisms and evaluate novel therapies.
Animal Models of Osteogenesis Imperfecta (OI)
At our core, we specialize in the precise engineering of advanced mouse models for osteogenesis imperfecta (OI), leveraging state-of-the-art gene editing and transgenic technologies to deliver unmatched genetic accuracy and model fidelity.
- oim Mice
- Heterozygotic G610C Mice
- Brittle IV (Brtl) Mice
- Jrt Heterozygous Mice
- Heterozygous Abnormal Gait 2 (Aga2) Mice
- Heterozygous Col1a1±365 OI Mice
- Crtap Mice
- IFITM5 Transgenic Mice
Featured Animal Models
| Model Name | Bmp1-Flox Mice |
| Model Type | Genetically Engineered Mouse Model (GEMM) |
| Modeling Method | Conditional Knockout |
| Sales Status | Embryo Cryopreservation |
| Detailed Description | These mice carry loxP sites flanking Exon 3-6 of Bmp1 gene. When crossed with a Cre recombinase-expressing strain, this strain is useful in eliminating tissue-specific conditional expression of Bmp1 gene. |
| Applications & Therapeutic Areas | This model is primarily applied in skeletal research and bone disorders, particularly for investigating the role of BMP1 in collagen fibrillogenesis and bone mineralization, with therapeutic relevance to osteogenesis imperfecta (OI) and other connective tissue diseases. |
Case Study-Col1a1 Mutant Mouse Model (C57BL/6Jseal/seal)
Model Introduction
To investigate the pathology of osteogenesis imperfecta (OI), this study utilized a mouse model generated by N-ethyl-N-nitrosourea (ENU) mutagenesis on a C57BL/6J mouse background. This procedure creates a recessive mutation (named seal) in the Col1a1 gene. This mutation disrupts normal collagen splicing, resulting in a typical OI phenotype in homozygous (seal/seal) mice, characterized by skeletal fragility and deformity.
Methodology
- Animal Model: C57BL/6Jseal/seal mutant mice and wild-type (WT) littermate controls on a C57BL/6J background were used.
- Phenotypic Analysis Methods:
- Skeletal Growth: Femur length was measured as a primary indicator of overall bone growth and development.
- Bone Resorption: Serum levels of type I collagen α1 chain C-terminal telopeptide (CTX) were quantified. CTX is a key biomarker for osteoclast activity and bone resorption.
- Bone Collagen Content: Hydroxyproline content was evaluated in demineralized bone hydrolysate to determine the total amount of collagen within the bone matrix.
Phenotypic Analysis & Results
The C57BL/6Jseal/seal homozygous mice exhibited key pathological features associated with osteogenesis imperfecta (OI):
- Impaired Skeletal Growth: Compared to wild-type (WT) mice, the femurs of C57BL/6Jseal/seal mice were significantly shorter (Fig.2A), indicating restricted skeletal development.
- Reduced Bone Resorption: Serum CTX levels in C57BL/6Jseal/seal mice were significantly reduced by approximately 65% (Fig.2B), demonstrating suppressed steady-state bone resorption (osteoclast activity).
- Decreased Collagen Content: Analysis of bone samples revealed that the hydroxyproline content in C57BL/6Jseal/seal mice was significantly lower than in WT controls (Fig.2C), confirming a reduction in total type I collagen in the bone matrix.
Fig.2 Phenotypic validation of the C57BL/6seal/seal mouse model. (A) Femur length. (B) Serum CTX levels. (C) Hydroxyproline content in bone tissue. Data are presented as mean ± SEM (n=5). *p < 0.05 vs. WT.
Conclusion
This case study validates the C57BL/6Jseal/seal mouse as a robust and reliable model for osteogenesis imperfecta (OI) research. The model successfully recapitulates critical disease hallmarks, including impaired bone growth, dysregulated bone remodeling, and defective collagen matrix. This makes it an ideal preclinical tool for investigating OI disease mechanisms and evaluating the efficacy of novel therapeutic strategies.
Contact Us
With our advanced animal models, Protheragen is committed to delivering comprehensive preclinical research services, encompassing pharmacodynamics (PD), pharmacokinetics (PK), and toxicology studies to thoroughly evaluate the safety and efficacy of your therapeutic candidates. If you are interested in our animal model development services, please do not hesitate to contact us for more details and quotation information.
Reference
- Alcorta-Sevillano N, Infante A, Macías I, et al. Murine animal models in osteogenesis imperfecta: The quest for improving the quality of life[J]. International Journal of Molecular Sciences, 2022, 24(1): 184.