Gerstmann-Sträussler-Scheinker Syndrome (GSS)

Gerstmann-Sträussler-Scheinker syndrome (GSS) is a rare, hereditary neurodegenerative condition falling under the umbrella of transmissible spongiform encephalopathies (TSEs). Our company stands out for providing extensive services, specifically in vaccine and therapeutic development, customized to meet the distinct requirements of researchers and scientists in rare infectious diseases like GSS.

Overview of GSS

The estimated occurrence of GSS ranges from 1 to 10 cases per 100,000 individuals, showing no noticeable differences based on gender. This condition is defined by the abnormal accumulation of prion proteins in the brain, setting off a cascade of neurological issues. Typically, GSS presents with an accelerated cerebellar syndrome marked by ataxia as an initial symptom, progressing rapidly into cognitive decline and additional neurological complications within a condensed timeframe, sometimes mere weeks or at most a few months.

Fig.1 A 39-year-old male diagnosed with GSS. (Shin, M., et al., 2023)

Pathogenesis of GSS

The inheritance pattern of GSS follows an autosomal dominant mode, with the frequent occurrence of a proline-to-leucine mutation at codon 102 (P102L) in the PrP gene. Mutations in this gene result in the creation of abnormal prion proteins that accumulate in the brain, interfering with normal brain function and driving neurodegeneration.

Vaccine Development for GSS

While there are currently no approved vaccines for prion diseases such as GSS, there have been advancements in vaccine development. Research in animal models has demonstrated that immunizing with recombinant PrP proteins or peptides can delay the onset or advancement of prion diseases. By utilizing modified or truncated PrP peptides, DNA vaccines, and bacterial/viral vectors as immunogens, it is possible to address the self-tolerance challenge.

Therapeutic Development for GSS

Several therapeutic strategies have been explored to inhibit prion replication and to clear abnormal prion proteins from the brain.

Our Services

Our company has cutting-edge infectious disease models, vaccine development frameworks, and therapeutic development platforms, and can provide a comprehensive suite of solutions. We excel in providing a seamless and integrated platform that caters specifically to the unique needs of those investigating rare neurodegenerative disorders like GSS.

Vaccine Development Platforms

Therapeutic Development Platforms

• Small Molecule Drug
• Therapeutic mRNA

• Therapeutic Antibody
• Cell Therapy

Infectious Disease Models

Animal models play a crucial role in studying diseases like GSS and understanding the underlying mechanisms. Our company provides various models of GSS to provide essential tools for researchers to investigate disease mechanisms and evaluate therapy approaches.

This model expresses a mutant form of the prion protein PrP, which is associated with GSS in humans and develops neurological symptoms, cognitive impairments, and prion protein deposition in the brain.

Optional Models: PG14 model; Tg(PrP-A116V) model, etc.

The one-stop platform for our company spans the entirety of the research stages, empowering researchers and scientists to hasten their investigations, make breakthroughs, and ultimately propel progress in comprehending and combating rare diseases like GSS. If you are interested in our services, we warmly welcome you to reach out for further details.

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