Jakob disease

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Prions-containing proteins (PrP) can be present in the bodies of both healthy people and animals. PrP embedded in transmittable material, on the other hand, has a complex composition and is resistant to proteases, which are enzymes in the body that break down proteins. Prions are communicable proteins with a lack of nucleic acids (Enger et al., 21). Nonetheless, prions that appear in virology textbooks have always captivated virologists.

Spongiform encephalopathy is a type of neurodegenerative disease caused by the miscoding of cellular prion proteins. Spongiform encephalopathies found in humans are categorized into three kinds: communicable, ancestral or hereditary, and intermittent, illustrious by what method the disease is attained initially. Usually, the pathogenic protein encoded as PrPC protein having a different conformation, known as PrPsc. In the modest case, the PrPSc alters the normal PrPC protein to form many duplicates of the pathogenic system.

The al form of the usual PrPC protein, showed some time back, exposed that it is mainly alpha-helical with tiny beta-strand contents. The structure of the PrPSc protein is shown to be elusive since it forms combinations and the amyloid fibrils (Gendoo, 16). It has been established that the PrPSc protein contains extra beta-strand contents than the usual protein; however how this asset would result in the prion replication was not revealed clearly. Research has found more on the structure of the prion due to the frequent practice of technological usage.

However, the structural form of the PrPSc protein has been identified by the cryo-electron microscopy which helped in the image reconstruction. This protein was cleansed from the transgenic mice encoded to create a form of the PrPSc protein which is not fixed to a cell membrane, which is under-glycosylated. The protein is shown to causes illness in the mice but is further consistent and forms the febrile plaques, permitting gentler sanitization methods.

The structural form of this PrPSc protein shows that it contains two entwined fibrils that are likely to comprise of series of the repeated beta-strands or some rungs which are called beta-solenoid. The structure gives some clues on how the pathogenic prion protein alters any normal PrPC into a PrPSc. The lower and the upper rungs of the beta-solenoids are the likely the start points for the hydrogen-bonding with the later PrPC molecules found in the numerous proteins with the beta-solenoids, then they are choked up to avert the propagation of the beta-sheets (Tycko, 64). When added to some of the fibrils, the results would aid in the process of recruiting the extra proteins, and these chains lengthen.

Many authors have noted that these molecular connections that control the prion templates, including the hydrogen-bonding, are charged and these hydrophobic interactions, the aromatic piling, and the steric restraints play critical roles in the DNA replication. The structural form of the PrPSc protein creates a mechanism for the prion replications through the combination of extra molecules into the budding beta-solenoid.

2. Discuss How the Disease Is Transmitted From Person To Person

The prion protein can trigger the normal proteins in the brains to fold unusually. A prion disease affects both people and the animals and these prions are occasionally spread to people via the septic meat products. The most usual form of prion disease which affects individuals is the Creutzfeldt-Jakob disease (CJD) (Llewelyn et al., 418).

The flawed proteins are transmitted by the contaminated and garnered human brain that produces electrode implants, Dural grafts or the corneal grafts which may show up in the patient in the form of the sCJD sporadic variant. In this hereditary manifestation, a genetic transfiguration occurs on the PrP and PRNP gene variants. About fifteen percent of the CJD situations often het considered as emanating from ancestry.

The onset of the Creutzfeldt-Jakob disease presumably occurs immediately after the consumption of the human growth hormones extracted from the pituitary glands of infected persons who had previously succumbed to the Creutzfeldt–Jacob illness. Since the documented occurrence of the disease in relation to the use of human growth hormones is relatively scantly and the data insignificant in light of modern scientific research, the probability for such an occurrence in itself still remains high.

Imperatively, it remains scientifically and biologically plausible that humans can get the disease through the consumption of materials from the infected animals with some bovine forms of this disease. The suspected condition of the occurrence so far has been the vCJD with the case in Canada and the UK. However, there is fear that is based on the animal studies which show that consumption of beef or some beef products having prion elements may also lead to the development of the classic CJD. When the BSE materials infect humans, the subsequent disease can be called new variant CJD (nvCJD).

Cannibalism has been associated to be a transmission device for the abnormal prions, which cause the disease called as kuru. The disease was once established primarily among some children and women belonging to a tribe found in Papua New Guinea (Will, 261). Though the men from this tribe ate a body of the dead and seldom got the infection, their women and the children that ate the fewer anticipated body portions, like the brains, were far more probable than the men to get the kuru from diseased tissues.

Blood Transfusion

A report present by the Lancet medical paper indicated that the vCJD is likely to be transmitted through blood transfusion. This finding upset the healthcare administrators due to a larger widespread of this disease that may occur sooner. A blood examination for a vCJD infection can be probable but cannot be accessible for the selection blood donation. A significant limit exists to protect some blood supply. Many countries have shown up to the prevention of the blood donation from individuals that have the virus to limit the transmission of the disease in the world.

Sperm Donations

The risk of CJD at some point in the American history caused the FDA to indefinitely ban sperm donation because of fear of human transmission. It was evident that the transmission of the disease was probably through the transmission of sperms from an infected person to a healthy one. Although the research was clear concerning the transmission of the disease, it did not state if it could be transmitted by sexual contacts.

3. Discuss the Relationship with Animal Prion Disease

Prion disease is common in the animals. The disease has different forms that are probable to cause death to the animal. The transmission methods of the disease are not far from those that evident in the human beings. The communicable prion diseases in animals are branded by the CNS spongiform changes, the gliosis and the accrual of disease-related methods of prion protein (PrPd) (Collinge, 317). Particularly among the ruminant’s prion ailments, the extensive ranges of the morphological natures of the PrPd removals are established in connotation with the neurons and the glia. When the light tiny patterns of the PrPd accretions are connected with the sub-cellular structures, the intracellular PrPd co-localizes by the lysosomes whereas the non-intracellular PrPd accretion co-localizes with the cells membranes and other extracellular spaces. The Intracellular lysosome PrPd can be the N-terminally condensed, and then the sites at which PrPd molecules are cleaved depending on the strain and the cell type. Some associates well with neurological diseases in mice.


Scrapie is a form of the TSEs. It occurs in goats, sheep, and moufflons. Similarly, prion infections, clinicopathological phenotypes of the scrapie differ on the prion stress and the animals' hereditary background. Clinical signs include behavior changes, impaired vision, incoordination, hyperexcitability, ataxia, and tremors. Strong pruritus is a common symptom that leads to loss of wool by through scraping and rubbing resulting to a characteristic chewing response from the animal once the dorsum is injured (Ungchusak, 335). Neuropathological symbols include the spongiform vacuolation and the removal of the PrPSc amyloid signs from the central nervous system. PrPSc is primarily detectable in the human nervous system, the tonsils, lymph nodes, spleens, nictitating membranes. Other spots include the placentas, muscles, distal ileums and the proximal colons.

Transmissible Mink Encephalopathy (TME)

TME is considered an infrequent TSE of the farmed mink, which is a minor semi-aquatic wild animal raised by several nations for the production of its fur. In 1947, TME was recognized in Minnesota and Wisconsin. The oral contamination of minks by the classical the BSE agents caused TME, nonetheless, animals were passive rather than being aggressive.

The disease is shown to transmit from one animal to another by feeding on the dead animal that had been infected (Young et al., 583). An animal that is carnivorous is at a high risk of viral attack because their teeth are mostly the carriers of the virus which breeds and is transported to the blood stream of the animal hence causing death.

4. Discuss the Difficulties Encountered In Sterilizing Contaminate Equipment

The sterilization of contaminated equipment involves a number of challenges. In most cases, the health practitioners have often suffered the complexity of the tool which is shown to have increased with time. From the decades back, the tools were made of glass and steel which were simple to clean, with the advancement in technology, the tools have become complex making it difficult to clean.

The process involved in the cleaning procedures of the tools is often dangerous to the health of the medical personnel making them not to undergo many risks in the cleaning process thus unable to sterilize them well (Schell, 2945). The cases are reported are characterized by the use of less advanced equipment by some hospitals that are less advanced. The incredible growth of the slightly intrusive surgeries in the world, however, conveyed elastic endoscopes which are passed via tiny notches to penetrate the body of the patients. Gadgets have become smaller, further specified and multifaceted, having moving parts and tiny holes. Manufacturers have turned to tools like the plastic, tungsten, and polymers. Complicated contemporary instruments transformed surgery, but then they have confirmed problematic to clean. The tiny interior channels are clogged with hidden tissues and blood.

A Central sterile tech plays an important duty in ensuring that fresh devices pass the surgery sites, but only New Jersey needs a tech that is proficient, although a contemporary push by the IAHCSMM and other state organizations (Webb, Sidney and Vall-Spinosa, 707). The use of incompetent tools in the cleaning mechanism is also a great challenge to the health practitioners. The insufficiency of the tools in the hospitals also play a great role in hindering proper cleaning of the tools; tools are not granted enough time to undergo the sterilization process. There are also a few individuals who are professionals in the field but are not motivated to perform well hence their work become faulty and raise difficulties in the process of dealing with the tools. An improper following of the instruction during the sterilization procedures often lead to difficulties in the cleaning process. The occurrence of new diseases in the world that have not been invented often create fears in the operators who become worried due to the health complications that might arise from the cleaning process of the tools

Works Cited

Collinge, John. "Variant creutzfeldt-jakob disease." The Lancet 354.9175 (1999): 317-323.

Enger, Eldon D, et al. Concepts in Biology. McGraw-Hill, 2012.

Gendoo, Deena. Bioinformatic Sequence and Structural Analysis for Amyloidogenicity in Prions and Other Proteins. 2012.

Llewelyn, C. A., et al. "Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion." The Lancet 363.9407 (2004): 417-421.

Schell, Daniel J., et al. "Contaminant occurrence, identification and control in a pilot-scale corn fiber to ethanol conversion process." Bioresource technology 98.15 (2007): 2942-2948.

Tycko, Robert. Molecular Structure of Amyloid and Prion Fibrils: Insights from Solid State Nmr. Henry Stewart Talks, 2008.

Ungchusak, Kumnuan, et al. "Probable person-to-person transmission of avian influenza A (H5N1)." New England Journal of Medicine 352.4 (2005): 333-340.

Webb, Sidney F., and Arthur Vall-Spinosa. "Outbreak of Serratia marcescens associated with the flexible fiberbronchoscope." Chest 68.5 (1975): 703-708.

Will, Robert G. "Acquired prion disease: iatrogenic CJD, variant CJD, kuru." British Medical Bulletin 66.1 (2003): 255-265.

Young, S. K., et al. "Microwave sterilization of nitrous oxide nasal hoods contaminated with virus." Oral surgery, oral medicine, oral pathology 60.6 (1985): 581-585.

January 05, 2023



Biology Zoology

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