A Significant Unmet Need

While the incidence of prion diseases is estimated at 1–2 new cases per million people each year, an approximate 1 in 5,000 deaths may be attributed to these disorders.

In humans, the vast majority of prion diseases occur sporadically, which means we do not understand why these individuals are susceptible. Approximately 10% of cases are inherited, and result from a mutation in the prion protein gene. Only a small fraction of these diseases arise from exposure to infectious prions, which can be transmitted through oral consumption or medical procedures.

Disease Manifestation

Chronic Wasting Disease

Due to their infectious nature, prion diseases are closely monitored in animals where they occur naturally. A prominent example is Chronic Wasting Disease (CWD), which has expanded dramatically in deer and elk populations across North America, Europe, and Asia over the past 50 years. Because cervids are part of the human food chain, CWD is a global concern. The risk that it could infect humans is a reminder of the urgent need for prion therapeutics.

The graph shows the historical global case numbers of prion diseases over a 27 year period (1993-2020). These historical numbers significantly under-represent the real numbers due to a lack of diagnostic tools and established surveillance programs. The relatively small diagnoses of prion diseases in countries with large populations like China indicate the lack of diagnostic competencies in these countries.

Over a 9 year period from 2011-2020, the data suggests that a total of 1138 cases of prion diseases are diagnosed each year. With no treatments available, this diagnosis is a death sentence with a rapid progression to fatal illness. Heprion is motivated to change this reality, by addressing this critical unmet societal need.

Prevalence Data