Gerstmann-Sträussler-Scheinker Syndrome (GSS)

Gerstmann-Sträussler-Scheinker syndrome (GSS) is an unusual, genetic, neurodegenerative disorder that can be classified as transmissible spongiform encephalopathies (TSEs). Our company is distinguished by the offering of a variety of solutions, more accurately in vaccine and therapeutic development, tailored to fit specific needs of researchers and scientists working with rare infectious diseases such as GSS.

Overview of GSS

The prevalence of GSS is between 1 and 10 cases per 100,000 population and does not show major differences according to sex. This ailment is characterized by the pathological deposition of prion proteins in the brain which triggers a chain reaction of neurological disorders. Generally, GSS appears to develop with an accelerated cerebellar syndrome with ataxia as the first sign and steeply advances to cognitive decline and additional neurological complications within a reasonably short period occasionally of some weeks or at most several months.

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

Pathogenesis of GSS

The GSS follows the autosomal dominant inheritance pedigree with the most common mutation seen being a proline to leucine alteration at position 102 which is P102L in the PrP gene. The variants of this gene cause modification of prion protein into abnormal forms which get deposited in the brain leading to functional disruption of the normal structure and cell death.

Vaccine Development for GSS

At present, there appear to be no licensed vaccines against prion disorders such as GSS. However, vaccine development has progressed over the years. Studies in animal models have shown that animals with recombinant PrP proteins or peptides can postpone the commencement or progress of prion diseases. The self-tolerance issue can be solved by using modified or shortened PrP peptides, DNA vaccines, and bacterial/viral vectors as immunogens.

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 possesses state-of-the-art models for infectious diseases, and platforms for vaccine and therapeutic development, thus enabling us to offer an integrated suite of solutions. We provided an exceptional and all-encompassing platform for GSS researchers, through which all of their problems are solved in a single setting.

Vaccine Development Platforms

Therapeutic Development Platforms

• Small Molecule Drug
• Therapeutic mRNA

• Therapeutic Antibody
• Cell Therapy

Infectious Disease Models

Animal models are indispensable in the study of diseases such as GSS and offer insight into the pathological mechanisms. We provide various models of GSS to enable researchers to study the pathophysiology of the disease and test out the therapies.

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.

Our company's comprehensive research platform enables researchers and scientists to work and progress at a faster pace. These services can make discoveries and contribute to the therapy regarding GSS and other rare diseases. 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.