Prion disease pathophysiology in central nervous system

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Prion diseases: about series

Nanobot Medical Animation Studio launched a series of educational movies on molecular biology. In the first video, “Prion diseases medical animation”, they explained the pathophysiological mechanism of some prion diseases.

First of all, the company used very sophisticated software to display the transformation processes, distribution, and aggregation of prions. The credit especially goes to the ePMV and autoPACK plugin. CellPACK is a specialized version of autoPACK that packs biological components together. Its function is to pack molecules into cells with biologically relevant interactions to populate massive cell models with atomic or near-atomic details. Components of the algorithm pack transmembrane proteins and lipids into bilayers; globular molecules in compartments defined by the bilayers (or as exteriors); and fibrous components like microtubules, actin, and DNA.

The first video is Prion diseases medical animation

A prion in the Scrapie form (PrPSc) is an infectious agent consists of protein in a misfolded form. It may lead to CJD appearance. Prions cause severe fatal neurodegenerative diseases by aggregating extracellularly within the central nervous system to form plaques known as amyloid. As a result, it disrupts the normal tissue structure. This disruption has a form of “holes” in the tissue with resultant spongy architecture due to the vacuole formation in the neurons. Other histological changes include astrogliosis with the absence of an inflammatory reaction. The incubation period for all prion diseases is relatively long (5 to 20 years). Most noteworthy, once symptoms appear, the disease progresses rapidly, leading to brain damage and death. Symptoms include convulsions, dementia, ataxia (balance and coordination dysfunction), and behavioral or personality changes. The video describes some features of the most common prion diseases related to humans.

  • Misshapen brain proteins are the etiological factor of Creutzfeldt-Jakob disease (CJD). CLD is a degenerative brain disorder.  In early stages, symptoms are often alike to patients with dementia, making CJD hard to diagnose. It is a rare disease that affects approximately one in a million people worldwide and leads to death.
  • Beef products are another cause of prion disease in humans called variant Creutzfeldt-Jakob disease (vCJD). Furthermore, Bovine spongiform encephalopathy (BSE) or, more commonly, “mad cow” disease is a form of this disease observed in cows.
  • Next example of human prion disease is kuru. It was discovered in the South Fore tribe in Papua New Guinea. The illness transmitted when tribe members fed on the flesh of infected people during cannibalistic funeral rituals.





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