Pandora's Box of Cannibals-Kuru and Ruan Virus

The protein misfolding cycle amplification technology (PMCA) strongly supports the protein hypothesis that PMCA can amplify the number of PrPSc by ultrasonically circulating infectious PrPSc and healthy brain homogenate
.
That is, PrPSc can convert normal PrPC, and the process grows exponentially
.

In transmission experiments, the commonly used route of infection is intracerebral vaccination, as Gedosek did to gorillas.
Because the concentration of PrPC in the brain is high, prions can replicate quickly
.
But this is not a way that would be done in the natural environment, except for iCJD cases (exposure to prion-contaminated neurosurgical materials, electrode probes or cadaveric dural transplantation)
.
Usually, prions enter the host through peripheral pathways and reach the brain after overcoming numerous obstacles
.

Like Kuru disease, prions are obtained by eating food contaminated by prions.
After entering the human body through the mouth, the infectivity of prions in the gut-associated lymphoid tissue (GALT) increases, and then the prions will pass through the peripheral nerves.
Reach the nervous system and gradually move towards the brain
.

People's understanding of prion diseases continues to deepen
.
Kuru’s epidemiological model shows that human diseases can be transmitted through peripheral routes such as oral administration.
Not only this, but other misfolding disorders with certain characteristics of TSE also have new ideas, such as Alzheimer’s disease.
It has been reported that PrPC may act as a receptor for b-amyloid oligomers or promote the formation of AZ amyloid plaques
.

One of the main characteristics that distinguishes TSE from other neurodegenerative diseases caused by protein misfolding and aggregation is their infectivity between individuals of the same species and other species
.

Prion diseases can "jump out" from their natural host
.
But due to the existence of the species barrier, this process is not so simple, and the results are not all lethal
.

Measures taken to minimize the risk of spreading prion diseases must take into account many unusual features of these diseases: (i) a long incubation period, up to several years or longer; (ii) can cause extensive lesions in the brain; (iii) ) Significant resistance to prions
.

Although humans have acquired considerable knowledge in the process of fighting prions, there are still many but unexpectedly acquired prion diseases; although it is not easy to spread between species, they still need to maintain the possibility of zoonotic diseases.
Vigilance
.
Be prepared for the threats that it may pose to human health in the future
.

At present, there is no clear treatment that can delay or even reverse the process of prion disease, and the main thing is symptomatic treatment
.
If you have epilepsy, use anti-epileptic drugs such as phenytoin and carbamazepine; if you have muscle cramps, you can use low-dose clonazepam; if you have difficulty swallowing, you can consider an esophagus to help you eat; if you have hallucinations/delusions and other mental illnesses , Requires low-dose antipsychotic treatment
.

In addition to these passive treatments, there are also some new developments, such as anti-PrP antibody therapy, which is intended to block the conversion of PrPC; the role of siRNA therapy is to inhibit the expression of PrPC, thereby reducing the substrate that can be converted into PrPSc, thereby successfully inhibiting the disease Progress
.
For genetic prion diseases, combining genetic testing with brain imaging and body fluid PMCA development strategies can provide treatment and prevention help
.

references:

1.
Liberski PP, Gajos A, Sikorska B, Lindenbaum S.
Kuru, the First Human Prion Disease.
Viruses.
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PMID: 30866511; PMCID: PMC6466359.

2.
Marín-Moreno, A.
, Fernández-Borges, N.
, Espinosa, JC, Andréoletti, O.
, & Torres, JM (2017).
Transmission and Replication of Prions.
Prion Protein, 181–201.
doi:10.
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3.
Sikorska, B.
, & Liberski, PP (2012).
Human Prion Diseases: From Kuru to Variant Creutzfeldt-Jakob Disease.
Subcellular Biochemistry, 457–496.
doi:10.
1007/978-94-007-5416-4_17

4.
Brown K, Mastrianni JA.
The prion diseases.
J Geriatr Psychiatry Neurol.
2010 Dec;23(4):277-98.
doi: 10.
1177/0891988710383576.
Epub 2010 Oct 11.
PMID: 20938044.

*Disclaimer: This article is written by the author of Sina Pharmaceutical News.
The views represent the author, not the author