All blood has some form of prion protein in it
and detecting the difference between the infections and normal particles can be
difficult. However, the difference between the infections and non-infectious
versions of the prion particles is the way that the protein is created. Both
types of protein are made up of amino acids, but the conformation is different
in normal protein particles, which effects the properties of the protein cell
dramatically.
The normal cellular prions that are found in
the blood have an unknown function, but they are normally expressed in brain
tissues such as; the hippocampus, medulla, thalamus, cerebral cortex and
cerebellum neurons. There are a few hypotheses on what the function of the
prion protein is in the brain. Some believe that they help protect neurons
against cell death, while others feel that they help with synaptic function and
the immune system. These are all in theory, because the actual use has yet to
be detected, but researches know that they do, in fact, play a vital role in
the brains functioning, yet they are unsure if they are required for human survival.
The infections version on prion particles in
the blood is very difficult to destroy. It has a very stable conformation and
the normal methods for destroying prion particles have all failed against this
infections blood disease. When treated with radiation, strong chemicals, heat
and proteases, the particles did not break up at all. As these toxic prions
remain in the blood, they play a large role in Nero-degeneration, which cannot
be reversed and will only get worse overtime. Those who have destruction to
their neurons from the pRp S version will experience side-effects such as;
memory loss, motor deficiencies and changes to their behavior. Prion disease is
closely related to the disease TSE, which is also characterized as creating a
“spongy brain appearance”.
The way that infections prion particles spread
through the blood and into the brain is unknown by researchers. It is thought
that a “misfold” may occur during the creation of a normal healthy prion
particle, which creates the infections particles that run through the body in
the same manner as the non-infections particles. Prion disease starts off very
slow in the blood, but once it has reached peak levels it creates symptoms
quickly and will spread rapidly through the brain.
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