PRIONS & PRION DISEASES

Prions are a kind of abnormally folded forms of normal proteins found in the brain, and also induces abnormal folding in the normal form of proteins. Prions are responsible for fatal and transmissible neurodegenerative diseases in both animals and humans.

Prion diseases are collection of several diseases arise due to the infection of prions and thereby brain damage.

Major prion diseases found in humans are Creutzfeldt-Jakob disease (CJD),Variant Creutzfeldt-Jakob disease (v CJD), Fatal Familial insomnia (FFI), Gerstmann-Straussler-Scheinker syndrome (GSS), and Kuru.

How prion proteins enters/ forms ?

Prions can enter the brain through infection (potentially by eating infected food) otherwise they can arise from mutation in the protein encoded genes also.To date, nobody knows quite how this happens.

What causes prion disease?

The mode of action of prion is very different on comparing bacteria and viruses since they are simply proteins, Once prion protein enters, it will induce normal protein to fold in abnormal shapes and accumulate abnormally in neuron and cause damage, the progressive destruction of neuron will results brain damage. This will leads to memory impairment, personality changes, and difficulties with movement etc.

Risk factors for prion disease ?

– Family history/ Genetics

– Age (Sporadic prion diseases tend to develop in older adults)

– Consuming animal products that are contaminated with a prion (mad cow disease) can transmit a prion disease to you.

– Infection by contaminated medical equipment, on receiving contaminated corneas and nervous tissue etc.

Common Symptoms ?

– Difficulties with thinking,memory and judgement.

– loss of coordination

– trouble sleeping

– difficult or slurred speech

– Hallucinations and confusion

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