Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
Chronic thromboembolic pulmonary hypertension (CTEPH) is a condition caused when pressure in small blood vessels in the lungs increases abnormally. With our company's profound expertise in CTEPH research, we are well-equipped to offer tailored solutions and comprehensive support to facilitate your research process from CTEPH therapy development to therapy commercialization.
Introduction to CTEPH
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and potentially life-threatening condition characterized by high blood pressure in the small blood vessels of the lungs. This condition is caused by the presence of blood clots and scarring in the pulmonary arteries, leading to increased resistance to blood flow and elevated pressure within the pulmonary circulation. The incidence of CTEPH is estimated to be 5 cases per million.
Fig. 1 Chronic thromboembolic pulmonary hypertension and targeted therapy. (Mahmud, Ehtisham, et al., 2018)Pathogenesis of CTEPH
CTEPH is characterized by a multifaceted mechanism involving thrombosis, impaired thrombolysis, and fibrotic changes in the pulmonary arteries. It is commonly associated with incomplete resolution of acute pulmonary embolism, leading to persistent organized thrombi within the pulmonary vasculature. These retained thrombi undergo fibrotic remodeling over time, resulting in pulmonary artery stenosis and obstruction.
Fig. 2 Pathophysiology of CTEPH. (Jung, Mi-Hyang, et al., 2022)Targets of CTEPH Therapy
Endothelin Pathway
Endothelin-1 (ET-1) is a potent vasoconstrictor that plays a central role in regulating vascular tone and remodeling. Elevated ET-1 levels are observed in individuals with CTEPH, suggesting a role in the development of pulmonary hypertension. Endothelin receptor antagonists may serve as potential therapeutic options for CTEPH.
Nitric Oxide (NO) Pathway
Nitric oxide is a vasodilator that promotes relaxation of smooth muscle in blood vessels, thereby improving blood flow. In CTEPH, impaired NO production and signaling lead to vasoconstriction and increased pulmonary vascular resistance. NO donors or phosphodiesterase 5 inhibitors are being investigated as potential therapeutics for CTEPH.
Our Services
Drawing upon our deep expertise in biotechnology and extensive experience in the industry, our company offers all-encompassing solutions for diagnostic and therapeutic research dedicated to CTEPH.
- CTEPH Diagnostic Development Services: For rare diseases such as CTEPH, our company offers diagnostic development services. We are dedicated to assisting you in developing advanced diagnostic tools to ensure accurate and timely detection.
- CTEPH Therapeutic Development Services: Our services extend beyond diagnostics, and we are also committed to the development of novel therapeutics for rare diseases. Our company provides a wide range of services for the development of small molecule drug, cell therapy, gene therapy, therapeutic antibody, therapeutic peptide, and therapeutic protein, to facilitate the effective management of CTEPH.
- CTEPH Animal Model Development Service: To support the preclinical research and development of CTEPH therapeutics, we offer animal models development services to facilitate your pharmacokinetics study and drug safety evaluation.
Animal Models of CTEPH
| Induced Models | ||
| Chemical induction models utilize substances that promote thrombus formation or impair thrombolysis. One such compound is monocrotaline, which is frequently used in rodent models. Monocrotaline administration leads to endothelial damage, thrombosis, and subsequent pulmonary vascular remodeling. These models help in studying the progressive fibrotic changes and hemodynamic alterations seen in CTEPH. | ||
| Genetically Engineered Models | ||
| Genetically engineered models involve the manipulation of specific genes associated with thrombosis or vascular remodeling. By targeting these genes, researchers can induce CTEPH-like phenotypes in animals. For instance, genetically modified mice with deficiencies in coagulation factors or fibrinolytic proteins have been utilized to investigate the role of these molecules in CTEPH development. | ||
| Optional Models |
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| Surgery Models | ||
| Surgical models involve the induction of pulmonary embolism through surgical interventions. For instance, in the rat model, autologous blood clots are introduced into the pulmonary circulation, leading to the formation of organized thrombi. This approach allows researchers to mimic the persistent thrombi observed in human CTEPH individuals. | ||
| Optional Species | Mice, Rats, Dogs, Pigs, Non-Human Primates (Monkeys), Others | |
No matter what stage of research you are at, we can provide you with corresponding research services. If you are interested in our services, please feel free to contact us for more details and quotation information for related services.
References
- Mahmud, Ehtisham, et al. "Chronic thromboembolic pulmonary hypertension: evolving therapeutic approaches for operable and inoperable disease." Journal of the American College of Cardiology 71.21 (2018): 2468-2486.
- Jung, Mi-Hyang, et al. "Clinical presentations and multimodal imaging diagnosis in chronic thromboembolic pulmonary hypertension." Journal of Clinical Medicine 11.22 (2022): 6678.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.