Sunday, January 9, 2011

prion Mad Cow Disease Creutzfeldt Jakob disease

What is a prion , What is Mad Cow Disease , What is Creutzfeldt–Jakob disease:

We all know about bacteria’s , viruses and fungi which causes serious infectious diseases in humans and animals , do you know about a protein molecule possessing same virulence , or activity just similar to bacteria’s and viruses .

Lets have a look at such complex protein molecule capable of producing symptoms of a virulence / infection in human or animal infected with it.

Its known as prion , are misfolded protien , it do not contain any RNA or DNA , as against other infectious microorganisms which multiply in their number using genetic information stored in to genes in DNA or RNA and to control and develop drug resistance or antibiotic resistance against incoming drugs or antibiotics a natural mechanism of their survival in an infected substrate .

Prion is capable of affecting brain and nervous tissue in human and animals, and can cause serious disease conditions like degenerative neurological disorder (brain disease) human and animals, a type of spongiform encephalopathy which is also a transmissible form, in humans it is called as Creutzfeldt–Jakob disease.
Animals infected with proin develop Bovine spongiform encephalopathy (BSE ) also known as Mad Cow Disease , In humans it is called as Creutzfeldt–Jakob disease.
Individual affected with proin develops in to Creutzfeldt–Jakob disease which result in to spongy degeneration in the brain and spinal cord .

Symptoms Creutzfeldt–Jakob disease ,  include rapid and progressive memory loss a state of dementia which also result in to changes in personality and individual develops hallucinations. Impaired Speech, changes in gait, rigid posture, and seizures , (myoclonus) jerky movements and (Ataxia) a state of impaired balance and coordination dysfunction.

Constituents of Proin :
Proin are made up of proteins , basically of abnormally folded PrP proteins , PrP proteins otherwise are also normal constituents of human and animal body , PrP in normal human individuals are in a state of normal folded pattern of proteins(orientation) where as PrP in a prion are in the state of abnormaly folded protein chains.

Role of PrP proteins in human body : It is belived that PrP proteins may be responsible for maintaining long term memory and may have a role in maintain a normal neurological functions, which is still to be investigated.
Mode of action of Prion :- Up on transmission in to a healthy individual Prion initiate a series of chain reactions , which result in to formation of abnormal folding in to otherwise normal PrP proteins in humans and animals body .

Transmission of proin to human is believed to be through

1.Eating food contaminated with tissues or consuming tissues of proin affected animals.

2. Through use of contaminated harvested human growth hormone (HGH) products and Immunoglobulins (IVIG)

3. Blood transfusions , as the UK was most affected with this disease a in there is ban in UK for blood donation by a individual who had received a blood transfusion since January 1980 , there is a ban for manufacturing of blood fractions like albumin in UK since 1999 by using blood of individuals from UK.

4. Heredetory : The disease becomes hereditary through a mutation in the gene for PrP , PRNP in an individual affected with disease.

In USA blood donation is prohibited for an individual who has spent three months in UK or total of five years or more of civilian travel in European countries.

Also see

Sterilization / destruction of proin / Decontamination procedures:
Proins are resistant to heat, radiation proteases, and formalin treatments as renaturation to some extent from denatured state may occur easily. Hence a combination of chemical hydrolysis of proin protein combined with moist heat sterilization is most effective method of decontamination of.

Also see sterlity testing in sterile dosase form

1. Autoclaving by immersing in a pan containing 1N NaOH and heat in a gravity displacement autoclave at 121°C for 30 minutes further rinse and clean with water and then perform regular sterilization processes.

2. Autoclave at 121°C for 1 hour heat in a gravity displacement autoclave , or in a porous-load (134°C) autoclave for 1 hour with a prior treatment in 1N NaOH or sodium hypochlorite solution (20,000 PPM of available chlorine) for 1 hour, further rinse and clean with water and then perform regular sterilization processes.

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