Accumulation of abnormal prion proteins in the brain characterizes neurodegenerative disorder which can be progressive and fatal. The consequence of such accumulation leads to gradual nerve cell damage that leads to cell death. We understand how complex prion-related disorders are and provide one-stop solutions to scientists and researchers working in this field.
Overview of Prion Diseases
Prion diseases, which are more commonly referred to as transmissible spongiform encephalopathies, are neurological degenerative disorders that touch on both human and animal populations. They have an exceedingly long incubation time which means they can take many years to develop. In humans, prion diseases can arise from random occurrence factors, genetic causation, and acquired factors. These diseases could also be transmitted in multiple ways including ingesting infected tissue, genetic mutations, spontaneous development of the disease, or through iatrogenic transmission.
Fig.1 Important mutations of genetic prion diseases. (Nafe, R., et al., 2023)
Pathogenesis of Prion Diseases
Prion diseases are caused by the pathological processes of the change of normal cellular prion protein (PrPC) into an abnormal and pathogenic form referred to as PrPSc. While the mechanism involved in this transformation is still not completely understood, it is thought to include the misfolding of PrPC to a conformational state enriched in beta sheets. The increasing load of PrPSc marks the formation of insoluble protein aggregates which initiates a cascade of neurodegenerative changes in the brain including neuronal devastation, gliosis, and the formation 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 firm’s services have the backing of highly skilled personnel and the most advanced technological infrastructural base that enables us to undertake a wider spectrum of tasks including - pathogen and host studies, developing diagnostics, vaccines, and therapeutic interventions, the development of animal models, and preclinical research that advances drug development for infectious diseases.
Types of Prion Diseases
Therapeutic Development Services of Prion Diseases
Vaccine Development of Prion Diseases
Animal Models Development of Prion Diseases
Animal models are important for understanding the pathogenesis, transmission, and therapeutic of prion diseases. Our firm has an extensive range of animal models of prion diseases which will enable the researchers to study such diseases from several perspectives such as disease mechanisms, progression, and therapeutic options.
Our team is comprised of experts in their respective fields who specialize in providing high and reliable solutions pertaining to life sciences so that researchers and scientists would have been in a better position to make decisions that could accelerate the ongoing research of prion diseases and their cure.
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.
