A boy afflicted with kuru disease
Source:Wikipedia, Author:Liberski PP,CC BY 3.0

Earlier, it was ludicrous to speculate that virus contained no nucleic acids. The nucleic acids are known to be macromolecules capable of storing information and as well show the genetic information stored in all cells and viruses. Traditionally, the nucleic acids are of two distinct type; DNA and RNA. However, scientists believe that no virus will survive without carrying any genetic information which enables them to duplicate. This assumption was challenged when Stanley B. Prusiner (American biochemist and neurologist) revealed an infectious virus which he named prion in 1982. Lately, prion certainty was not solely discerned until the pioneering work carried out by Stanley Prusiner.

what is prion

Prion is understood to be an infectious virus, negligibly distinct from other virus in eras of not possessing a nucleic genome.
According to researchers, prion, mostly observed in the cells of the brain, is a neuro degenerative fetal disease induced not by virus nor bacterium but due to abnormal conformation of protein in the brain. The specific needs of protein in the brain cells is to generate neurotransmitters in which chemical carriers are being transported in order to make effective communications in the brain. However, it is unsound to infer that prion disease will stem from deficiency of proteins in diet instead insufficient of protein in food will inflict your system not to create enough neurotransmitters hence depression and aggressive behavior will result because of low level of dopamine and serotonin production.
Back to our subject, prion was found out to be the chief cause of neurodegenerative diseases, affecting both humans and animals. Like I said before, prion is tinier than virus and it requires high quality electron microscope to view and lacks hereditary materials or genome but it can replicate.

Naturally, all humans have prion protein (Proteinaceous infectious Particle), which is made up of two distinct types; the normal protein called the PrPc and the abnormal protein called the PrPsc.
Scientists assume that when infectious PrPsc is introduced into the body, possibly, normal PrPc is overpowered by the deformed PrPsc in prion bind, hence the shape of PrPc is deformed too and changed Into PrPsc therefore stimulting an exponential rise of the abnormal PrPsc protein.
Researchers glued to the above theory as an evidence to support normal protein (PrPc) as the cause of prion disease in the brain cells since it can alter it's shape to diseased protein when encountered with the abnormal infectious protein (PrPsc). Though this assumption has not been fully accepted.

Diseased proteins in the cell infected with prions are capable of causing dementia disorder like Creutzfeldt Jakob disease(CJD) and Kuru in human, bovine spongiform encephalopathy also known as ''mad cow" disease in animals.
According to Prusiner, traditionally prion can stimulate in different significant ways.

''A remarkable feature of prion disease is that they can arise in three different ways. They can occur spontaneously, or be triggered by infection, or occur as a consequence of hereditary predisposition".

From the above perception, we can acknowledge that slight alteration or mutation in gene effected with prion can be transmitted from mother to a newly developed baby. Scientists have indicated that the possibility of new variant CJD,(another form of Creutzfeldt Jakob disease caused by BSE) prion being transmitted to a newly formed fetus is through the placenta. This hold that any parent harboring or incubating the prion disease for a long time can possibly pass it to her potential child. United Kingdom has recorded a few cases whereby a prion disease in human was transmitted through blood transfusion, so donating blood from areas known to been infected heavily with prion disease have been prohibited.

Like I said before, prion is believed to cause severe neurological disorders like ;

• kuru and Creutzfeldt Jakob (found in human)
• Bovine spongiform Encephalopathy BSE( mad cow disease)
• Scrapie in sheep.

Kuru disease was first discovered among the people of Papua New Guinea.

Brain affected with prion
Source:Wikipedia, Author:Liberski PP, CC BY 3.0
It was reported that the disease was transmitted to individuals who were involved in consuming the cooked brain of a dead member during a ritual funeral. According to information, Kuru known as transmissible Spongiform encephalopathy(TSE). In 1957, kuru disease was tagged as a deadly epidemic due to high rate of cases observed. Between 1957-1961 and from 1961- 2004, the number of death issues recorded were more than 35000. It was noted that minimum of 200 people died every year during the time of the epidemic. Symptoms of the disease identified in sufferers with kuru were shivering and movement disorder, this prompted people of New Guinea to name the disease "kuru", meaning shivering and abnormal movement. Evidently, this prion disease was mostly common among the adult females because the cannibal rite was left predominantly for the women than men and this enhanced easy transmission of the disease to their children.

When Carelton Gadjusek took research about the disease, he found out that kuru was possibly evoked by slow virus,Creutzfeldt Jakob Disease (CJD) is also caused by slow virus. From research, CJD is a rare rapid transmitting disease known to be inflicted by prion, affecting people under the age of 68. Scientists have not reported the disease transmitted through touching an infected person or air borne or by blood contact and sexual relationship but it was understood that transmission was via inheritance or eating diseased brain or nervous tissue.
Researchers have revealed that Creutzfeldt Jakob disease destroys the brain cells rapidly when infected thus causing the brain to form numerous small sponge holes. Affected Prion protein, when conformed to abnormal isoform in the brain cell will result to fatel neurodegenerative disorder like insomnia, depression, difficulty to move and speak, coma, blindnes etc.
Another form of erratic CJD is variant CJD (vCJD), mostly found among the youths, that is people below the age of 30 according to Centers for Disease Control. This type is rare and it is recognized to be transmitted through consuming cattle infected with Bovine spongiform encephalopathy(BSE).

It is strongly believe that cows are affected with BSE when they are feed with scrapie, that is sheep meat and bone. Incubation of vCJD in a person can not be easily noticed and it is being count in terms of years, this is to say that individual affected with vCJD will not exhibit any sign for so many years. The origin of BSE is yet to be discovered. From statistics, United Kingdom was recognized to had high number of cattle infected with BSE in 1986. Preventive laws implemented by Animal and Plant Health Ispection Service of the United States Department of Agriculture was

"to placed restrictions on the importation of live ruminants such as cattle, sheep, goat and certain ruminants products from countries where BSE was known to exist".

Diagnosis of prion disease involves imaging and taking note of the brain electrical actions. Highly specialized devices like EEG scan, MRIs and biopsy are employed to scan and detect affected areas or abnormal signs in the brain.
It has been documented that people affected with prion disease hardly survive. Individual affected with prion is being assisted with medication in order to lessen and slow down the rate in which the disease advances, despite the certainty that prion disease can never be cure.

References

•CJD
•Creutzfeldt Jakob disease
•What is CJD
•Prion-CJD
•Causes of CJD
•Human prion disease
•Bovine Spongiform Encephalopathy
•Kuru disease
•Kuru
•About Prions
•Prion basics
•Prion disease
•Prion diseases arise in three different ways
•Signs You're Not Getting Enough Protein
•Nucleic Acid
•Noble prize
•Prions
•What is prion
•Prion disease