# Pathogenesis, Transmission and Detection of Zoonotic Prion Diseases

> **NIH NIH P01** · UNIVERSITY OF TEXAS HLTH SCI CTR HOUSTON · 2022 · $2,576,111

## Abstract

ABSTRACT
Prion diseases are a group of infectious neurodegenerative disorders affecting humans and other
mammals, which are transmitted by an unconventional proteinaceous infectious agent termed prion. CWD
is so far the only prion disease of wild animals; it is highly contagious and the exact origin, prevalence, and
mechanisms of transmission remain incompletely understood. The progressive increase of CWD prevalence in
the USA has likely resulted in a substantial contamination of the environment with infectious prions and may
lead to new prion diseases in animals that share the habitat of cervids. The disease has been rapidly
expanding geographically and now affects 27 states in USA, three Canadian provinces, South Korea and
Northern Europe, including Norway, Sweden and Finland. The risk of transmission of CWD to other animal
species or to humans is unknown and surveillance methods to detect the infection in non-cervid species are
limited.
This Program Project has received continuous funding for the past 11 years, which has enabled us to
implement a large and complementary set of unique techniques, model systems, reagents, personnel and
expertise crucial to understand the CWD problem. In the last competitive review, we received a perfect
score of 10 and reviewers identified many strengths and “not any significant weakness”. The progress
during this cycle has been stellar, with many important discoveries and 118 high profile publications. The
main goal of this iteration of the Program Project will be to study the molecular and cellular mechanisms
responsible for prion replication and prion strain diversity in CWD. We will study the generation, mutation and
evolution of CWD prion strains, the inter-species transmission potential of CWD (with particular focus on its
zoonotic potential) and the role of the environment on CWD transmission. This Program Project aims to
address some of the most important questions in CWD research, including the possibly origin of CWD, the
mechanism and routes of transmission of the disease among animals, the natural strain diversity of CWD, the
atomic resolution structure of infectious prions, the evaluation of the zoonotic potential of CWD, the factors
controlling the cervid/human species barrier, the role of the environment on prion transmission and the
development of efficient procedures to detect infectious CWD prions. To achieve these goals, this Program
Project assembles a group of highly accomplished scientists in the prion field, particularly experts on CWD
transmission, who have access to the relevant models, techniques, samples, resources and expertise to
provide a complementary and comprehensive investigation of the problem.

## Key facts

- **NIH application ID:** 10332504
- **Project number:** 2P01AI077774-11
- **Recipient organization:** UNIVERSITY OF TEXAS HLTH SCI CTR HOUSTON
- **Principal Investigator:** CLAUDIO SOTO
- **Activity code:** P01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2022
- **Award amount:** $2,576,111
- **Award type:** 2
- **Project period:** 2008-08-15 → 2027-02-28

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10332504

## Citation

> US National Institutes of Health, RePORTER application 10332504, Pathogenesis, Transmission and Detection of Zoonotic Prion Diseases (2P01AI077774-11). Retrieved via AI Analytics 2026-05-24 from https://api.ai-analytics.org/grant/nih/10332504. Licensed CC0.

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