Prion Diseases Drug Discovery Service

In the field of neurological disease research and drug development, Creative Biolabs is a well-deserved expert with years of project experience. Based on our advanced platform, we can help clients accomplish different tasks in all stages of drug development.

Introduction of Prion Diseases

Prion diseases are a rare group of neurodegenerative disorders caused by abnormally folded proteins in the brain, especially the misfolding of prion proteins (PrP). All prion diseases are caused by one molecular mechanism, that is, changes in the conformation of the prion protein (PrP) encoded by the PRNP gene from its intrinsically folded form (PrPC) to a self-replicating misfolded form (PrPSc). Prion diseases can affect both humans and animals, leading to a gradual decline in brain function. There are approximately 300 new cases of prion disease every year in the USA. Prion diseases have a long incubation period, usually several years. Once symptoms appear, they can get worse quickly.

Since prion diseases always present similar symptoms to other neurodegenerative disorders, postmortem brain biopsy is the only way to confirm a prion disease. What's more, magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) testing, and electroencephalography (EEG) would be helpful for prion disease diagnosis. There's currently no cure therapy for prion diseases. Scientists at Creative Biolabs are working on prion disease research and potential treatment development.

Fig.1 The same protein might undergo structural changes which lead to the formation of different abnormal protein conformers. (Moda, et al., 2019)

Types of Human Prion Diseases

• Creutzfeldt-Jakob disease (CJD)
• Variant Creutzfeldt-Jakob disease (vCJD)
• Fatal Familial insomnia (FFI)
• Gerstmann-Straussler-Scheinker syndrome (GSS)
• Kuru

Common Symptoms of Prion Disease

• Difficulty thinking, remembering, and judging
• Personality changes
• Visually impaired or blind
• Difficulty speaking or slurring
• Difficulty sleeping
• Myoclonus
• Confusion or disorientation

Drug Discovery against Prion Diseases

Since conversion from the normal cellular isoform of PrP (PrPC) to aberrant prion protein (PrPSc) is the underlying pathogenesis, Creative Biolabs has developed a series of in vitro screening approaches for drug discovery against prion diseases. For example, Real-Time Quaking Induced Conversion (RT-QuIC) and Protein Misfolding Cyclic Amplification (PMCA) are novel techniques to test compounds rapidly in a reproducible way. Reliable preclinical screening methods are an important factor in identifying suitable drugs, and all identified molecules have been screened using cell-based assays and in vivo murine models. For the special needs of customers, we can also develop customized models and methods.

Creative Biolabs is a leading service provider that focuses on neuroscience research. We are confident in offering the best products and services for our clients all over the world. If you are interested in our services and products, please do not hesitate to contact us for more details.

Reference

1. Moda, F.; et al. Novel screening approaches for human prion diseases drug discovery. Expert Opinion on Drug Discovery. 2019, 14(10): 983-993.