Transmissible Spongiform Encephalopathies

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

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.

The word encephalitis is derived from two Greek words. Enkephalos, which means brain, and itis, which means inflammation. Encephalitis was defined by Sir John Burton Cleland, a pathologist and naturalist. When he defined it, it was named ‘Australian X disease’.

Affecting 1 in every 18,000 people, Adrenoleukodystrophy (ALD) is a genetic disease that destroys the myelin sheath surrounding a brain neuron. A brain neuron is an essential cell body that is responsible for muscle contractions and ultimately, our ability to move. Adrenoleukodystrophy is a devastating genetic mutation that affects X-chromosomes in both males and females. However, because males only have one X-chromosome, the outcome is catastrophic.

     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).

Whenever we are sick, we go to the doctor in hope of getting answers and a cure but what if there is no cure to your disease. Amyotrophic lateral sclerosis (ALS) and chronic traumatic encephalopathy (CTE) are two horrible progressive degenerative disease. ALS is a nervous system disease that causes muscle weakness and impacts all physical functions except the brain. Therefore, the individual is well aware of how his or her body is deteriorating. CTE on the other hand affects the brain, not the body, so those who suffer from it may say or do things they would normally not do such as physically harming a loved one. Medical research shows that both diseases are caused by brain injuries such as brain trauma and concussions. Head trauma, which

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.

Tay-Sachs disease, (also known as GM2 gangliosidosis or hexosaminidase A deficiency), is a fatal autosomal recessive genetic disorder caused by insufficient activity of the enzyme beta-hexosaminidase A. It is very rare, found more prevalently among certain populations, like those with Eastern European, (Ashkenazi Jewish) heritage (Bethesda, 2014), and usually results in death by the age of four. The purpose of this essay is to illustrate the pathology and inheritance patterns of this genetic disorder, and provide the molecular base, prognosis, and possible treatments.

What is Mad Cow Disease? Mad Cow Disease "is a normally extremely rare neurological disorder that affects the central nervous system of an animal and a human. The disease infested brain of the animal or human is slowly, progressively, and severely damaged. It has been categorized as a disease, which falls into the category of a disease known as Spongiform Encephalopathy. Having Bovine Spongiform Encephalopathy is an over-dramatized way of specifying that one has diseased brain tissue which is; spongy, porous, and no

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

In the beginning, they thought that the practice of cannibalism was the reason for this disease. After many years of study and research they learned that was so. Michael Alper, Carlton Gajdusek, and Shirley Linderbaum were directly involved and responsible for discovering the source, mapping its progression, and finding the first victim of Kuru. Through their Study and research Linderbaum determined that the first person afflicted in this tribe was back in the early 1900. Alper figured out that it was being transmitted through the mortuary eating of the brain in particular and Gajdusek and the others discerned that Scrapie, Creutzfeldt-Jakob disease and Kuru the way they transmitted were closely related and they believe that the first person came down with Creutzfeldt-Jakob disease which is a spontaneous change in the brain, they died and were consumed. They proved that it was transmittable from humans to primates and from animals to humans. Later, Biochemist Stanley Prusiner gave the named a new pathogen in over 100 years, Prion, a protein that duplicates itself by recruiting other protein replicating itself and infecting the brain, organs and vascular system. What made this pathogen special and very different was that it has no know antibodies, it was incredibly small nearly 1000 times smaller than any infecting

Generally, a sponge is equated with the soaking or consumption of liquids. Nevertheless, this is not representative of this disease or its effects on the human brain. This progressive and debilitating disease maintains many unknowns, and patient outlook is equivalent to lethal injection. Creutzfeldt-Jakob’s Disease (CJD) is a rare, degenerative, invariably fatal brain disorder, derived from transmissible spongiform encephalopathy caused by prions. Prions occur in a normal state, which are harmless proteins found in the body’s cells, and also in an infectious form that causes disease. Harmless forms of prion proteins have the same sequence of amino acids, but the infectious forms of protein have a different folded shape than normal proteins.

Transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases that are thought to be caused by the misfolding of prion proteins. Prions are able to replicate in the absence of nucleic acids. TSEs include: scrapie, bovine spongiform encephalopathy, Creutzfeldt-Jakob disease, kuru, Gerstmann-Straussler-Scheinker disease, and Fatal Familial Insomnia. They can affect many different animals, including humans. Currently, there are no ways to diagnose, treat, or cure TSEs, as much more research is needed before these diseases are completely understood.

The amyloid plaque is the cause of deadly genetic degeneration of a person’s nervous system, mainly in the neurons of the brain. The brain tissue has holes in it and looks like a sponge. Prions cause Creutzfeldt Jacob disease, which is rare neurological human disease. Prions are also responsible for causing mad cow disease in cattle (Norrby, 2011).