Sign Prion Disease

Creutzfeldt-Jakob Disease: It happens when a prion protein misfolds itself, thus causing a “domino effect” which unfolds itself causing a malfunction.

Proteinaceous Infectious Particles, commonly known as Prions, are extremely rare misfolds of the protein PrPc, which cause fatally neurodegenerative diseases, and are theorized to be infectious only by the protein itself (U.S National Library of Medicine, 1998). This “protein-only theory” is still heavily debated today, as some scientists deny the theory, and there isn’t a significant amount of evidence on each side to qualify the theory or disprove it (Soto, C. 2011). The base “Prion” protein is encoded in the gene PRNP, while being non-infectious. Prions are most commonly found in human prion diseases, but they can also be in other animals in the form of Mad Cow Disease and Chronic Wasting Disease, classified as Bovine Spongiform Encephalopathies

Although serious, CJD is rare. Worldwide, there is an estimated one case of Creutzfeldt-Jakob disease diagnosed per million people each year, most commonly in older adults.

Humans have to deal with many different diseases and the ones most disliked are the ones with no cures. Like cancer, transmissible spongiform encephalopathies have no cure, but they are more rare. These diseases are prion diseases which cause the brain to deteriorate. Prions are proteins that sometimes behave like viruses, which mean that they should have some form of nucleic acid, but since they don’t, they cause abnormalities. The nervous system contains many normal prions, but when an abnormal prion comes along, it transforms all the normal prions into abnormal ones. Bovine spongiform encephalopathy is found in cattle, but it can be transmitted to humans.

     Mad Cow Disease, scientifically referred to as (BSE) Bovine Spongiform Encephalopathy, is a disease that affects those humans who eat the meat from infected cows. Mad Cow Disease is one of several fatal brain diseases called (TSE) Transmissible Spongiform Encephalopathy. (USDA) There was evidence of a new illness resembling the sheep disease scrapie. It was technically named BSE but quickly acquired the mad cow tag because of the way infected cattle behave. (CNN) In 1997, there was an award given to Stanley Prusiner, for concluding that a distorted protein called a prion was responsible for Mad Cow Disease, noted the long incubation period made it difficult to distinguish (Bryant).

In bovine spongiform encephalopathy (BSE), the disease is caused by the misfolding of proteins that cause proteins and peptides to develop a fibrillary structure. The PrPc is a correctly folded prion and the misfolded form is called PrPSc. BSE occurs when the normal PrPc come into contact with the toxic PrPSc and the normal prion takes on the shape of the PrPSc. The normal chaperones are unable to convert the PrPSc back to the normal form. The PrPSc now takes on the role of chaperone and the conversion of PrPc prions continue over and over. PrPSc, now being hydrophobic avoids the water of the inner cell and begin to accumulate and form plaques along the neuronal cell membranes. The aggregation of the prions on the cell membrane eventually lead to cell death which produces the sponge-like appearance in the brain of cattle infected with BSE (Thompson, 2014).

Variant Creutzfeldt-Jakob disease (vCJD) is a rare and fatal human neurodegenerative condition falling under the category of Transmissible Spongiform Encephalopathy (TSE) because of having characteristic of spongy degeneration of the brain that it causes and its ability to be transmitted. First it was described in the United Kingdom in March 1996 and it has been connected with exposure to a TSE of cattle known as Bovine Spongiform Encephalopathy (BSE) sometimes called Classical BSE, having been reported first in 1986 in the United Kingdom.

Abnormal changes happen in the brain, and it appears as a sponge-like holes and gaps, causing deterioration in the areas of the brain. This can turn into spongiform degeneration that results in the progressive neurological and neuromuscular symptoms that are associated with CJD. This disorder is a result from a mutation in the gene that regulates the production of the human protein or direct transmission with abnormal protein in infected brain tissue. Scientist believe that there is a transmissible agent that is responsible for causing CJD. This is a slow virus, since it can take many years from exposure to the appearance of

The U.S. Department of Agriculture (USDA) has tested hundreds of thousands of cattle for BSE. Researchers believe that the infectious agent that causes mad cow disease is an abnormal version of a protein normally found on cell surfaces, called a prion. For reasons still unknown, this protein becomes altered and destroys nervous system tissue (brain and spinal cord). There exists strong epidemiologic and laboratory evidence for a causal association between a new human prion disease called variant Creutzfeldt-Jakob disease (vCJD) that was first reported from the United Kingdom in 1996 and the BSE outbreak in cattle (http://www.cdc.gov). According to The National Creutzfeldt-Jakob Disease Surveillance Unit, by June 2014 it had killed 177 people in the United Kingdom, and 52 elsewhere. This essay will focus on the possible causes, effects, and treatment for this

The aggregation of prion proteins and their transmissibility from one cell to another has been shown to be evident (Cushman et al.; Goedert et al. 2010), therefore strongly suggesting that these events may play a role in pathogenesis for many diseases, including both AD and PD. While none of these diseases is infectious in an identical way as

In both my body and your’s there are these things called prions which occur normally in humans but when folded incorrectly, they are harmful to the body. These incorrectly folded prions then go on and alter other normal prions by actually changing the structure of the prion’s alpha helical regions into beta pleated sheets. If these altered prions are in your brain, they build up in your neurons and cluster together with each other eventually causing neuron loss and other brain damage. This continued brain damage and neuron loss leads to holes in the brain giving it the consistency of a

In addition to the cervid species in which chronic waste disease can occur in wide variety of other animals. These other animals include monkeys, sheep, cattle, prairie voles, mice, and ferrets. Environmental transmission has been linked to contact with infected bodily fluids and tissues. Once the prions are in the environment chronic waste disease prions may remain infectious for many years. Thus, Decomposition of the disease carcasses, infected gut piles left by hunter, as well as urine, saliva, feces, and antler velvet of infected individuals that are deposited in the environment.

Mad Cow Disease Bovine spongiform encephalophathy ( BSE), which is mainly known as mad cow disease has infected the society and has put the lives of individuals at risk. This was very frightening to the people that ate meat during that time. The terrified people would not even want to eat hamburgers at MacDonald, steak at restaurant and much more. What also alarmed the people was the fact that there was a possibility of getting the human form of mad cow disease. This was called variant Creutzfeldt-Jakob disease (vCJD) which is through tainted blood, but this was only a theory which

Each one mentioned above is very different form the other. Each one also has various sub-types of the condition. Today approximately one in ever fifty thousand children born are affected by the disease. In today’s general population nine in one million suffer from this disease. Most people who have this disease pass away at a very young age. The oldest know person living with this condition was Tabatha Nichols of Hastings Michigan. She was twenty-four years old when she passed away in her

Prions are a type of protein found naturally in the brain and other regions of the central nervous system. The diseases associated with