Paget's Disease of Bone
Paget's disease of bone is a chronic bone disorder characterized by abnormal bone remodeling, leading to enlarged and weakened bones. This condition primarily affects skeletal sites like the skull, spine, pelvis, and long bones of the arms and legs. Through cutting-edge technology and a deep understanding of the disease mechanisms, we offer a comprehensive range of services to support your research at every stage.
Overview of Paget's Disease of Bone
Paget's disease of bone is a chronic focal disorder of bone remodeling with an incidence of approximately 0.2 / 10,000., affecting one or more bones that are typically enlarged and deformed. One of the key features of Paget's disease is the high level of bone remodeling activity, with both bone resorption and formation processes occurring at an accelerated rate. This results in the characteristic enlargement and deformation of affected bones.
Fig.1 The major skeletal locations affected by Paget's disease of bone. (Banaganapalli, B., et al., 2023)Pathogenesis of Paget's Disease of Bone
Genetic and environmental factors play a significant role in the pathogenesis of Paget's disease of bone. Over the last two decades, researchers have identified germline mutations in multiple genes and common variants that predispose individuals to this disorder. Some of the genes implicated in Paget's disease include SQSTM1, TNFRSF11A, and VCP. These genetic abnormalities contribute to an imbalance in bone remodeling processes, leading to excessive bone resorption by osteoclasts and subsequent abnormal bone formation by osteoblasts.
Fig.2 Updated pathogenetic model of Paget's disease of bone. (Gennari, L., et al., 2022)Diagnostics Development of Paget's Disease of Bone
In addition to imaging examinations, there is a significant relationship between some specific markers of bone resorption and disease activity, which can be used as biomarkers to evaluate extension and the metabolic activity of the disease.
- Bone-specific alkaline phosphatases (B-ALP)
- Procollagen 1 Intact N-Terminal Propeptide (P1NP)
- Serum carboxy-terminal peptide of type 1 collagen (sCTx)
- Total alkaline phosphatase (t-ALP)
- Urinary amino-terminal peptide of type 1 collagen (uNTx)
- Urinary carboxy-terminal peptide of type 1 collagen (uCTx)
Therapeutics of Paget's Disease of Bone
Our Services
Our company specializes in studying rare diseases like Paget's disease of bone. From animal models that mimic Paget's disease to advanced therapeutic development platforms, we provide the tools and resources needed to accelerate research and drive progress in the field.
Therapy Development Platforms
Animal Models of Paget's Disease of Bone
Animal models of Paget's disease of bone provide a valuable tool for researchers to better understand the disease and develop new therapeutics for individuals suffering from this condition. Our company offers a variety of genetically engineered animal models of Paget's disease of bone to accelerate your research.
| Genetically Engineered Models | ||
| This model involves manipulating genes associated with bone remodeling by transgenic or gene editing technology such as CRISPR/Cas9 to induce Paget-like bone changes. | ||
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| Optional Species | Mice, Rats, Dogs, Non-Human Primates, Others | |
Our commitment to excellence and passion for rare disease research makes us a trusted partner for you, we provide services including pharmacokinetics and biosafety evaluation to advance the understanding of this complex disorder and develop innovative therapeutic approaches.
If you are interested in our services, please feel free to contact us for more details and quotation information of related services.
References
- Corral Gudino, Luis "Paget's disease of bone: 1877-2023. Etiology, and management of a disease on epidemiologic transition." Medicina clinica 161.5 (2023): 207-216.
- Gennari, Luigi et al. "Update on the pathogenesis and genetics of Paget's disease of bone." Frontiers in cell and developmental biology 10 (2022): 932065.
- Banaganapalli, Babajan et al. "Paget's disease: a review of the epidemiology, etiology, genetics, and treatment." Frontiers in genetics 14 (2023): 1131182.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.