Prion diseases are progressive, fatal neurodegenerative disorders characterized by the accumulation of abnormal prion proteins in the brain. This accumulation leads to the gradual damage and eventual death of nerve cells. Companies that specialize in infectious diseases, like ours, offer a range of comprehensive services to aid researchers and scientists working in the field of prion diseases.
Overview of Prion Diseases
Also known as transmissible spongiform encephalopathies, prion diseases are rare and fatal conditions that affect both humans and animals. They have a long incubation period and are considered universally fatal. Human prion diseases can arise from three main sources: sporadic factors, genetic predispositions, and acquired sources. The transmissibility of these diseases can occur through various means, such as ingesting contaminated tissues, inheriting genetic mutations, experiencing spontaneous occurrences, or through iatrogenic transmission.
Fig.1 Important mutations of genetic prion diseases. (Nafe, R., et al., 2023)
Pathogenesis of Prion Diseases
The pathogenic processes of prion diseases involve the transformation of normal cellular prion protein (PrPC) into an abnormal, pathogenic form (PrPSc). Although the exact mechanism of this conversion is not yet fully understood, it is believed to involve the misfolding of PrPC into a beta-sheet-rich conformation. The burgeoning accumulation of PrPSc heralds the genesis of insoluble protein aggregates, triggering the neurodegenerative alterations in the brain, encompassing neuronal devastation, gliosis, and the birth of microscopic vacuoles.
Fig.2 Agents targeting prion proteins. (Shim, K. H., et al., 2022)
Vaccine Development of Prion Diseases
Fig.3 Progression of prion diseases.
| Prion vaccine components |
Antigens |
PrPC as target |
| PrPSc-specific antigens |
| Disease-specific epitopes |
| Structurally restrained epitopes |
| Vaccine formulation and delivery |
Adenovirus vectors |
| Lambda phage |
| Bacterial delivery |
Therapeutics Development of Prion Diseases
| Therapeutics Names |
Therapeutics Types |
Mechanism of Action |
Research Phase |
| Anle138b |
Chemical compounds |
Inhibit PrPSc oligomerization |
Phase I trials |
| GN8 |
Chemical compounds |
Block PrPSc generation |
Ready for clinical trials |
| Baicalein |
Natural compounds |
Inhibit JNK phosphorylation |
Preclinical research |
| scPOM-bi |
Antibody |
Restricted the conversion of PrPC |
Preclinical research |
| PRN100 |
Antibody |
Bind and stabilize PrPC |
Phase II trials |
Our Services
Our company is equipped with state-of-the-art facilities and a team of experts dedicated to providing a wide array of services to support research and development in infectious diseases. These services include pathogen and host research, diagnostics development, vaccine development, therapeutic development, infectious disease model development, and preclinical research.
Types of Prion Diseases
Therapeutic Development Services of Prion Diseases
Vaccine Development of Prion Diseases
Animal Models Development of Prion Diseases
Furthermore, animal models are instrumental in studying the pathogenesis, transmission, and potential therapeutics for prion diseases. Our company offers a diverse range of animal models of prion diseases to aid researchers in exploring various aspects of the conditions, including disease dynamics, progression, and potential therapeutic strategies.
Our team of highly skilled professionals is dedicated to delivering accurate and timely results, enabling researchers and scientists to make informed decisions and advance their studies, ultimately contributing to the progress in understanding and combating prion diseases.
If you are interested in learning more about our services or obtaining quotations, please do not hesitate to contact us for further information and assistance.
References
- Shim, Kyu Hwan et al. "Prion therapeutics: Lessons from the past." Prion 16.1 (2022): 265-294.
- Satoh, Katsuya. "CSF biomarkers for prion diseases." Neurochemistry international 155 (2022): 105306.
- Napper, Scott, and Hermann M Schatzl. "Vaccines for prion diseases: a realistic goal?." Cell and tissue research 392.1 (2023): 367-392.
All of our services and products are intended for preclinical research use
only and cannot be used to diagnose, treat or manage patients.
