What are Prions?

Prions meaning is an atypical form of usually harmless protein, found in the brain. It is responsible for several neurodegenerative diseases among humans and animals.

Moreover, prion’s course of action is unlike any bacteria or virus. The reason being, it is just protein lacking any genetic material. In case a misfolded protein enters a human body, it affects the correctly-folded protein to affect adversely.

Furthermore, in the early 1980s, Stanley B. Prusiner and his colleagues first identified the prions. Additionally, the term prion came from “proteinaceous infectious particle”. This misfolded protein enters a human or animal brain via infection. However, it can also arise from a mutation of the gene that encodes protein.

However, scientists have not fully understood the mechanism till date.

What is a Prion Infection?

Prion infection or transmissible spongiform encephalopathies (TSE) is a family of uncommon progressive neurodegenerative disorder. It affects the brain of both humans and animals. Additionally, prions are the causative agents of TSEs.

Notably, this abnormal folding of protein can result in brain damage, and this disease is usually fatal.

Types of Prion Disease

CJD: This disease is related to the ‘mad cow disease’. Individuals can inherit this illness or develop it suddenly. However, most cases of CJD are sporadic and seen in people around the age of 60.

Variably Protease-Sensitive Prionopathy (VPSP): This prion definition is also rare, and has similarities with CJD. However, here the affected protein is less sensitive to digestion. This form of prion is witnessed in individuals around the age of 70, with a family history of dementia.

Gerstmann-Sträussler-Scheinker Disease (GSS): This form is extremely rare and witnessed in people around the age of 40.

Kuru: This particular form of prion is mostly cured due to awareness. It appeared in New Guinea as a result of consuming human brain tissue contaminated with infected prions.

Fatal Insomnia: This is a rare form of insomnia. Moreover, it is hereditary; thus, there is no sporadic form of this disease.

Reasons for Prion Disease

The Reason Behind Prion Disease are –

  • Prion disease can be genetic. Individuals with a family history of this disease are at risk of developing the mutation.

  • Older adults suffer from sporadic prion disease.

  • Moreover, consuming contaminated animal products with an altered prion structure can transmit this disease.

  • Last but not least, using contaminated medical equipment is a major reason behind its spread. Notably, a medical procedure like dura mater graft or cornea transplant with infected tissue can transmit it also.

Symptoms of Prion Disease

Prion disease has a long incubation period, often years. However, when symptoms develop, they worsen progressively and at times rapidly. Some of the common symptoms of this illness are –

  • Changes in personality are the arrival of agitation, depression, etc.

  • Rapidly developing dementia

  • Disorientation

  • Uncontrolled muscle spasms

  • Insomnia

  • Losing coordination

  • Hallucinations

  • Fatigue

  • Difficulty in speaking

  • Blindness

Diagnosis of Prions Disease

To Diagnose Prion Disease, Doctors use Different Tests such as –

  • MRI, to scan the brain.

  • Electroencephalogram to analyse brain waves.

  • Collecting fluid samples from the spinal cord.

  • Blood test

  • Visual and neurological exams to diagnose vision loss and nerve damage.

Treatment of Prion Disease

There is no specific treatment available for this illness. However, the application of medicine can slow its growth.

Prions meaning suggests that it is a complicated disease and has no treatment available. As a result, you should learn more about its traits along with those of other rare diseases with the help of our detailed study material. Now you can also download our Vedantu app for convenient access to these study materials and online interactive sessions for enhanced clarity on these ailments.

FAQ (Frequently Asked Questions)

1. What is a Prion?

Ans. A prion is an unusual form of a typically harmless protein, found in the brain. This protein is responsible for various neurodegenerative diseases among humans and animals. However, it is not any virus or bacterial infection, rather just a protein malfunction.

2. What is Prion Biology?

Ans. Prion biology refers to the detailed study of this rather complicated disease which has a long incubation period and rapid spread, along with long term effects. Till date, no cure for this disease has been found. Medication can be used only for its control.

3. What are the Diseases Which are Caused by Prions?

Ans. Some of the prominent diseases caused by prions are CJD, kuru, fatal insomnia, variably protease-sensitive prionopathy and Gerstmann-Sträussler-Scheinker disease. Notably, this disease is sporadic and hereditary.