Related Experiment Video
Updated: Feb 12, 2026
Prion Safety Laboratory Swipe Test
Published on: February 14, 2025
A Practical Primer on Prion Pathology
Brian S Appleby1,2, Daniel D Rhoads1,3, Karin Mente4
1National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio.
Abstract:
Prion diseases comprise a group of transmissible degenerative encephalopathies resulting from propagation of a misfolded cellular protein of uncertain function. As is generally the case with rare diseases, lack of institutional experience compromises individual familiarity with the varying, and apparently protean, manifestations of prion diseases, both clinically and pathologically. Coupled with the documented transmissibility of these diseases both within and between species, the Centers for Disease Control and Prevention (CDC) has established the National Prion Disease Pathology Surveillance Center to both aid with diagnosis of prion disease and to survey the United States for evidence of zoonotic transmission. We have assembled this primer with the hope that our accumulated experience will enable the neuropathological community to help the CDC "save lives and protect people."
Insights
Prion diseases are rare, transmissible neurodegenerative disorders caused by misfolded proteins. This primer aids diagnosis and surveillance for prion diseases, including zoonotic transmission, to protect public health.
Area of Science:
- Neuropathology
- Infectious Diseases
- Rare Diseases
Background:
- Prion diseases are transmissible neurodegenerative disorders.
- Misfolded proteins propagate, causing cellular damage.
- Rare disease status limits clinical and pathological experience.
Purpose of the Study:
- To provide a primer on prion disease diagnosis and pathology.
- To aid the Centers for Disease Control and Prevention (CDC) in surveillance.
- To facilitate early detection and prevent zoonotic transmission.
Main Methods:
- Assembled accumulated experience from a national surveillance center.
- Focused on clinical and pathological manifestations.
- Emphasized disease transmissibility within and between species.
Main Results:
- The primer aims to improve neuropathological community's familiarity with prion diseases.
- Enhanced diagnostic capabilities are expected.
- Improved surveillance for zoonotic transmission is anticipated.
Conclusions:
- This primer will enhance the neuropathological community's ability to diagnose and manage prion diseases.
- It supports the CDC's mission to "save lives and protect people."
- Increased awareness and expertise are crucial for controlling prion disease spread.
Related Concept Videos
01:16Characteristics of Practical Op Amps
The ratio of differential gain to the common-mode gain is defined as the common-mode rejection ratio (CMRR). This ratio quantifies the ability of operational amplifiers (op-amps) to reject common-mode...
01:30Theoretical Foundations of Nursing Practice
Theories provide a perspective to assess patients' conditions and organize data and methods. They also assist in analyzing and interpreting information. They represent a...
01:28Equivalent Circuits for Practical Transformers
In a practical transformer, each winding exhibits resistance and leakage reactance. The...
03:03Amyloid Fibrils
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
01:59Lagging Strand Synthesis
There are several major differences between synthesis of the leading strand and synthesis of the lagging strand. 1) Leading strand synthesis happens in the direction of replication fork opening, whereas lagging strand synthesis happens in the...
01:32PCR