Paraneoplastic Pemphigus
Paraneoplastic pemphigus (PNP) is an uncommon autoimmune blistering disease that is intricately linked with malignancies. Our company has established itself as a leader in the field of drug development and therapy specifically tailored for autoimmune disorders and inflammatory conditions, including paraneoplastic pemphigus.
Overview of Paraneoplastic Pemphigus
Paraneoplastic pemphigus is an autoimmune blistering disorder that is often associated with underlying malignancies, most commonly lymphoproliferative neoplasms. It is characterized by the presence of autoantibodies that target desmosomal and hemidesmosomal proteins, such as desmoglein and BP180, leading to the destruction of cell-cell adhesion and subsequent blister formation. PNP typically presents with painful mucocutaneous lesions, including erosions, blisters, and ulcerations, which can involve not only the skin but also the oral cavity, esophagus, respiratory tract, and other organs.
Fig.1 Hematoxylin-eosin staining analysis of Paraneoplastic pemphigus (PNP). (McNally M. A., et al., 2021)
Drug Discovery and Development for Paraneoplastic Pemphigus
Corticosteroids, such as high-dose systemic corticosteroids, are considered first-line therapy for PNP. They help suppress the immune response and reduce inflammation, leading to a reduction in blister formation and mucocutaneous lesions. Therapeutics targeting specific components of the immune system have also shown efficacy in PNP therapeutics. Monoclonal antibodies, such as rituximab (anti-CD20 B-cell depleting monoclonal antibody), have demonstrated some success in PNP with underlying lymphoproliferative malignancies.
At our company, we are fully dedicated to the advancement of diagnostics and therapeutic solutions for paraneoplastic pemphigus. To gain deeper insights into the range of services we offer for therapy development, we warmly invite you to explore the provided link for comprehensive information.
Small Molecule Drug
Probiotics Therapy
Therapeutic Antibody
Cell Therapy
Gene Therapy
Therapeutics Development
Our Services
To expedite the progress of therapeutic development, our company places a significant emphasis on the creation of robust animal models and in vitro models that faithfully replicate the complex pathology of paraneoplastic pemphigus. These meticulously designed models serve as indispensable tools, enabling us to gain extensive insights into the underlying disease mechanisms, conduct comprehensive evaluations of potential drug candidates, and assess their efficacy in a controlled and reliable environment.
Neonatal Mouse Models
IgG from paraneoplastic pemphigus patient sera is administered intraperitoneally into neonatal BALB/c mice. The mice develop cutaneous blisters and erosions that closely resemble the clinical and histological features of human paraneoplastic pemphigus.
Autoreactive Cell Transfer Models
In addition to passive transfer of PNP autoantibodies, transfer of autoreactive immune cells has also been utilized to study the pathogenesis and therapies of PNP. Our Company has developed expertise in the transfer of autoreactive lymphocytes, such as T cells and B cells, into recipient animals.
By isolating and culturing primary keratinocytes, we can assess the effects of PNP-associated autoantibodies or immune cells on these cells. Our Company has expertise in generating primary keratinocyte models and optimizing culture conditions to maintain their functional and phenotypic characteristics.
Our company has the expertise to generate and optimize skin organoid models for PNP research. Moreover, we offer the development of multi-organoid systems that incorporate skin organoids along with other relevant organoids, such as oral mucosa or lung epithelium.
By employing these cutting-edge models, we are able to accelerate the development of novel and targeted therapeutic interventions for paraneoplastic pemphigus. In addition to the aforementioned range of services and models, we also specialize in tailoring customized solutions and developing disease models that precisely align with your distinctive requirements. Should our array of services capture your interest, we warmly encourage you to reach out to us without hesitation.
Reference
- McNally M. A., et al. "Paraneoplastic pemphigus manifesting in a patient treated with pembrolizumab for urothelial carcinoma." JAAD Case Reports 10 (2021): 82-84.