Related Experiment Video

Updated: Feb 11, 2026

Protein Misfolding Cyclic Amplification of Prions
10:12

Protein Misfolding Cyclic Amplification of Prions

Published on: November 7, 2012

20.2K

Rapid amplification of prions from variant Creutzfeldt-Jakob disease cerebrospinal fluid

Marcelo A Barria1, Andrew Lee1, Alison Je Green1

  • 1National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, Deanery of Clinical Medicine, The University of Edinburgh, Edinburgh, UK.

The Journal of Pathology. Clinical Research
|April 18, 2018
PubMed

Insights

A new seeding conversion assay rapidly detects prion protein in variant Creutzfeldt-Jakob disease (vCJD) patients. This highly sensitive test shows 100% accuracy for all vCJD cases, regardless of genetic prion protein gene (PRNP) codon 129 status.

Area of Science:

  • Neuroscience
  • Infectious Diseases
  • Genetics

Background:

  • Human prion diseases are fatal neurodegenerative disorders caused by misfolded prion proteins.
  • Variant Creutzfeldt-Jakob disease (vCJD) is a zoonotic form linked to bovine spongiform encephalopathy (BSE).
  • Asymptomatic vCJD carriers may be prevalent (1 in 2000 in the UK), and transmission via blood transfusion is a concern.

Purpose of the Study:

  • To develop a sensitive, reliable, and rapid diagnostic test for vCJD.
  • To detect disease-associated prion protein in vCJD patients.

Main Methods:

  • A rapid and highly sensitive seeding conversion assay was developed.
  • The assay amplifies disease-associated prion protein from brain and cerebrospinal fluid samples.
  • Amplification occurred over 48-96 hours.

Main Results:

  • The assay demonstrated 100% sensitivity and specificity for vCJD detection.
  • It successfully amplified prions from definite, probable, and possible vCJD cases.
  • The test is effective for patients with both methionine homozygous (MM) and heterozygous (MV) prion protein gene (PRNP) codon 129 genotypes.

Conclusions:

  • A rapid, sensitive, and specific diagnostic assay for vCJD has been established.
  • This assay can detect prion protein in vCJD patients across different genetic profiles.
  • The findings support the need for widespread vCJD screening.
Keywords:
Creutzfeldt-Jakob diseasediagnostic testprionprotein misfolding

More Related Videos

Sample Preparation for Endopeptidomic Analysis in Human Cerebrospinal Fluid10:23

Sample Preparation for Endopeptidomic Analysis in Human Cerebrospinal Fluid

Published on: December 4, 2017

9.4K
An Improved Method for Collection of Cerebrospinal Fluid from Anesthetized Mice06:40

An Improved Method for Collection of Cerebrospinal Fluid from Anesthetized Mice

Published on: March 19, 2018

66.7K

Related Concept Videos

Cerebrospinal Fluid01:21

Cerebrospinal Fluid

Cerebrospinal fluid (CSF) is a colorless liquid that flows around the brain and the spinal cord, playing a vital role in the protection, support, and overall function of the central nervous system (CNS). CSF production, circulation, and absorption are tightly regulated processes essential for the brain and spinal cord to function properly.
CSF Production
CSF is produced mainly in the choroid plexus, a network of capillaries and ependymal cells located within the ventricular system of the brain....
6.1K
RACE - Rapid Amplification of cDNA Ends02:35

RACE - Rapid Amplification of cDNA Ends

Rapid Amplification of cDNA Ends, or RACE, is one of the most effective methods to obtain a full-length cDNA from an mRNA sequence between a known internal region to the unknown sequence at the 5’ or 3’ end. The unknown region is cloned in the cDNA by a gene-specific primer that binds the known end, and a hybrid primer that attaches a predefined anchor sequence to the unknown end of the cDNA. The sequence in between is amplified by PCR with an anchor primer and a gene-specific...
7.3K
Histone Variants at the Centromere02:30

Histone Variants at the Centromere

Histone variants are the histone proteins with structural and sequence variations. These variants may be regarded as “mutant” forms that replace their canonical histone counterparts in the nucleosomes. Specific post-translational modifications on the histone variants enable further chromatin complexity and regulate tissue-specific gene expression. The most common histone variants are from histone H2A, H2B, and linker histone H1 families. However, several variants of histone H3...
5.1K
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining,...
12.0K
The Fluid Mosaic Model01:34

The Fluid Mosaic Model

The fluid mosaic model was first proposed as a visual representation of research observations. The model comprises the composition and dynamics of membranes and serves as a foundation for future membrane-related studies. The model depicts the structure of the plasma membrane with a variety of components, which include phospholipids, proteins, and carbohydrates. These integral molecules are loosely bound, defining the cell’s border and providing fluidity for optimal function.
179.5K
Fluid Pressure01:14

Fluid Pressure

In mechanical engineering, fluid pressure plays a critical role in designing systems that utilize liquid flow, such as hydraulic systems, pumps, and valves. When designing these systems, engineers must ensure they can withstand the forces created by fluid pressure to avoid damage or failure.
According to Pascal's law, a fluid at rest will generate equal pressure in all directions. This pressure is measured as a force per unit area, and its magnitude depends on the fluid's specific...
1.3K