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Idiopathic Pulmonary Arterial Hypertension (IPAH)

Idiopathic pulmonary arterial hypertension (IPAH) is a condition in which blood pressure in the pulmonary arteries increases. With our company's profound expertise in IPAH research, we are well-equipped to offer tailored solutions and comprehensive support to facilitate your research process from IPAH therapy development to therapy commercialization.

Introduction to IPAH

IPAH is a progressive and debilitating rare disease characterized by high blood pressure in the pulmonary arteries, which are responsible for carrying oxygen-depleted blood from the heart to the lungs. This condition leads to the narrowing and stiffening of the pulmonary arteries, making it difficult for blood to flow through them. As a result, the right side of the heart has to work harder to pump blood, leading to right-sided heart failure and potentially life-threatening complications if left untreated. The prevalence of IPAH ranges from 0.4 to 1.4 per 100,000 individuals.

Idiopathic Pulmonary Arterial Hypertension (IPAH)Fig. 1 The key abnormal pathways targeted in the pharmacological therapeutics of pulmonary arterial hypertension and the mechanism of action for contemporary drugs. (Lan, Norris SH, et al., 2018)

Pathogenesis of IPAH

The precise cause of IPAH remains unknown, hence the term "idiopathic." However, extensive studies have established the involvement of genetic factors, inflammatory processes, and endothelial dysfunction in the development of IPAH.

Genetic Factors

Genetic factors are one of the important causes of IPAH. Mutations in genes such as BMPR2, ALK1, EIF2AK4 and ENG have been identified in a subset of IPAH cases, indicating a genetic predisposition.

Inflammatory Process

Abnormal immune responses and dysregulation of immune cells contribute to the remodeling of pulmonary arteries. The release of pro-inflammatory cytokines and growth factors further promotes vascular cell proliferation and fibrosis.

Endothelial Dysfunction

Endothelial dysfunction in IPAH is marked by reduced vasodilator production and increased vasoconstrictor production. This imbalance disrupts pulmonary artery tone regulation, leading to elevated pulmonary arterial pressure and heightened vascular resistance.

Targets of IPAH Therapy

  • Vasoconstriction Pathways

Inhibiting vasoconstriction is key to developing therapies for IPAH. Prostacyclin analogs, such as epoprostenol, can promote vasodilation and improve exercise capacity in IPAH individuals. Endothelin receptor antagonists, such as bosentan, mibrisentan, and macitant, block vasoconstriction.

  • Endothelial Dysfunction

Restoring endothelial function is another important strategy. Phosphodiesterase-5 inhibitors (PDE-5 inhibitors), including sildenafil and tadalafil, promote vasodilation, inhibit smooth muscle cell proliferation, and improve exercise capacity by increasing cGMP levels.

  • Inflammatory and Immunity

Inflammation and immune dysregulation play a role in the pathogenesis of IPAH. Immunomodulatory therapies, such as rituximab, which target specific immune cells involved in the inflammatory response, may help reduce vascular remodeling and inflammation in individuals with IPAH.

Our Services

With years of extensive involvement in rare disease research, our company boasts a highly skilled team and vast expertise. We harness state-of-the-art technology to drive the development of innovative diagnostic tools, enabling early detection of IPAH. By establishing animal models and conducting in-depth investigations into IPAH pathogenesis and targets, we advance therapeutic drug development.

Our Research Platforms

Our Services

Animal Models of IPAH

Induced Models
By employing physical, chemical, or biological stimuli, we can induce pulmonary hypertension symptoms in animal models. For instance, animal models of IPAH have been created through methods such as chronic hypoxia, exposure to toxins, or infection with schistosomiasis.
Optional Models
  • Chronic Hypoxia-Induced Model
  • Sugen 5416 Induced Model
  • Monocrotaline Induced Model
  • Bleomycin Induced Model
  • Mitomycin‐c‐induced Model
  • Schistosomiasis Infection Model
Surgical Intervention Models
Invasive surgical methods in pulmonary hypertension models mainly include pneumonectomy, pulmonary shunt, and pulmonary artery binding (PAB). Among them, pneumonectomy and vascular shunting lead to an increase in residual pulmonary artery blood flow, which plays a key role in the occurrence of pulmonary hypertension.
Genetically Engineered Models
Numerous genes, including BMPR2, ALK1, EIF2AK4, and ENG, have been identified as susceptibility genes for IPAH. Leveraging advanced gene editing technologies like CRISPR/Cas9, our scientists can introduce IPAH-associated genetic mutations into animal models, enabling the replication of key features observed in human IPAH.
Optional Models
  • BMPR2 Mutation Model
  • ALK1 Mutation Model
  • EIF2AK4 Mutation Model
  • ENG Mutation Model
  • KCNK3 Mutation Model
  • S100A4/Mts1 Transgenic Model
  • 5-HTT Overexpression Model
  • IL-6 Overexpression Model
Optional Species Mice, Rats, Pigs, Dogs, Others

No matter what research stage 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 of related services.


  • Lan, Norris SH, et al. "Pulmonary arterial hypertension: pathophysiology and treatment." Diseases 6.2 (2018): 38.
  • Wu, Xiao‐Han, et al. "Experimental animal models of pulmonary hypertension: Development and challenges." Animal Models and Experimental Medicine 5.3 (2022): 207-216.

All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.

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