Understanding Prions

Simply put, prions are proteins gone rogue. These bad actors drive the progression of neurological diseases. Tracking prions is a sure-fire way to accurately diagnose Alzheimer’s, Lewy Body Dementia (LBD), Parkinson’s (PD), and other brain diseases. Amprion’s scientific strength is the amplification of prions.

What are prions?

To understand prions (aka misfolded proteins or prion-proteins), let’s first understand what normal proteins are.

Proteins are linear chains composed of building blocks called amino acids. Each linear protein chain folds naturally into a unique three-dimensional shape.

Some proteins can misfold into one or more alternative forms. Occasionally, these misfolded shapes may lead to disease. Under rare circumstances, the misfolded proteins may induce other normal proteins to misfold.  These self-replicating and toxic misfolded proteins are called prions (or prion-proteins).

Think of prions as protein origami gone awry.

Play Video

Watch this short video to see how prions fly under the radar and destroy our brains over decades without the body showing any symptoms.

Prion's role in neurological diseases

Brain diseases are prion-related.  At Amprion, we consider they exist on a spectrum called ParkinZheimer™.

The critical prion biomarkers in ParkinZheimer diseases include Abeta, Tau, and Synuclein.

How prions damage the brain

Once a normal protein misfolds, it becomes a prion and gains the ability to corrupt other normal proteins to misfold. One prion can multiply into billions over several years, forming a zombie prion army. They can move directly from cell to cell or travel through the blood or cerebral spinal fluid.

This prion army continues to spread, destroying neurons along the way. During this development stage, the patient has no physical symptoms or any loss of mental abilities.

This asymptomatic stage can last years or multiple decades, slowly damaging the brain without us knowing.

When we finally notice the loss of physical or mental abilities, we visit doctors for diagnosis.  This is already the late-stage because much of the brain has suffered irreversible damage.

Prions’ ability to replicate and proliferate on a massive scale plays a key role in the progression of ParkinZheimer diseases. Prions are virtually undetectable using traditional methods.

Once prion concentration reaches a certain threshold within a brain cell (or neuron), it causes cell death. Unlike most cells in the body, neurons cannot regenerate. This brings irreversible loss of mental and motor abilities.

Prion biomarkers in ParkinZheimer diseases

In Alzheimer’s, misfolded proteins cause plaque and tangles.

In Parkinson’s and LBD, prions form Lewy Bodies.

As observed under the microscope, plaque, tangles, and Lewy Bodies damage the brain in the following ways:

  • Prions are soluble nanoparticles that spread disease by moving between connected nerve cells.

  • Prions appear to disrupt normal protein synthesis and degradation within nerve cells leading to cell death.

  • At high concentrations, misfolded proteins tend to form large insoluble aggregates easily detected under a microscope.

Alzheimer’s biomarkers

So far, scientists have discovered various types of prion biomarkers associated with Alzheimer’s.  It’s worth noting that some diseases may show more than one prion biomarker. Most Alzheimer’s patients show these two prion types:

  • Abeta
  • Tau

Furthermore, among 20-40% of Alzheimer’s patients, we also find these two misfolded proteins:

  • Synuclein
  • TDP43

LBD and Parkinson’s biomarker

In  the brains of LBD and PD patients, only one biomarker is detected so far:

  • Synuclein

Prion MOST WANTED LIST:

ABETA, TAU & SYNUCLEIN.

We’re working hard to crack the code on how they function in the early stages. This is the key to unlocking cures. 

blog image - parkinson's diagnosis

PRION MOST WANTED LIST:

Abeta

Tau

 Synuclein

We’re working hard to crack the code on how they function in the early stages. This is the key to unlocking cures. 

What increases the risk for proteins to misfold?

Decades of research show several triggers causing normal proteins to misfold.

  1. Traumatic head injuries
  2. Genetics/family history
  3. Environmental factors

Prion research pioneers

For nearly two decades, our scientists have solved parts of the prion mystery and produced a voluminous body of peer-reviewed research papers. Every day, our team strives to better understand prions and formulate strategic plans to defeat them.

Here are some key questions we’re seeking to answer:

  • Why do normal proteins misfold?
  • When and where does this first happen?  
  • How do they spread within the brain?
  • What are the critical pathways driving the decline of cognitive and motor functions?
  • How do they disrupt intracellular processes to cause brain cell death?
amprion logo quote solid blue
The man with no imagination has no wings.
Scroll to Top
Share on facebook
Share on twitter
Share on linkedin