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Prions
A prion () is a misfolded protein that induces misfolding in normal variants of the same protein, leading to cellular death. Prions are responsible for prion diseases, known as transmissible spongiform encephalopathy (TSEs), which are fatal and transmissible neurodegenerative diseases affecting both humans and animals. These proteins can misfold sporadically, due to genetic mutations, or by exposure to an already misfolded protein, leading to an abnormal three-dimensional structure that can propagate misfolding in other proteins. The term ''prion'' comes from "proteinaceous infectious particle". Unlike other infectious agents such as viruses, bacteria, and fungi, prions do not contain nucleic acids (DNA or RNA). Prions are mainly twisted isoforms of the major prion protein (PrP), a naturally occurring protein with an uncertain function. They are the hypothesized cause of various TSEs, including scrapie in sheep, chronic wasting disease (CWD) in deer, bovine spongifor ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Scrapie
Scrapie () is a fatal, degenerative disease affecting the nervous systems of sheep and goats. It is one of several transmissible spongiform encephalopathies (TSEs), and as such it is thought to be caused by a prion. Scrapie has been known since at least 1732 and does not appear to be transmissible to humans. However, it has been found to be experimentally transmissible to humanised transgenic mice and non-human primates. The name scrapie is derived from one of the clinical signs of the condition, wherein affected animals will compulsively scrape off their fleeces against rocks, trees or fences. The disease apparently causes an itching sensation in the animals. Other clinical signs include excessive lip smacking, altered gaits and convulsive collapse. Scrapie is infectious and transmissible among conspecifics, so one of the most common ways to contain it (since it is incurable) is to quarantine and kill those affected. However, scrapie tends to persist in flocks and can also ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Transmissible Spongiform Encephalopathy
Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of progressive, incurable, and fatal conditions that are associated with the prion hypothesis and affect the brain and nervous system of many animals, including humans, cattle, and sheep. According to the most widespread hypothesis, they are transmitted by prions, though some other data suggest an involvement of a ''Spiroplasma'' infection. Mental and physical abilities deteriorate and many tiny holes appear in the cortex causing it to appear like a sponge when brain tissue obtained at autopsy is examined under a microscope. The disorders cause impairment of brain function which may result in memory loss, personality changes, and abnormal or impaired movement which worsen over time. TSEs of humans include Creutzfeldt–Jakob disease, Gerstmann–Sträussler–Scheinker syndrome, fatal familial insomnia, and kuru, as well as the recently discovered variably protease-sensitive prionopat ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Creutzfeldt–Jakob Disease
Creutzfeldt–Jakob disease (CJD) is an incurable, always fatal neurodegenerative disease belonging to the transmissible spongiform encephalopathy (TSE) group. Early symptoms include memory problems, behavioral changes, poor coordination, visual disturbances and auditory disturbances. Later symptoms include dementia, involuntary movements, blindness, deafness, weakness, and coma. About 70% of sufferers die within a year of diagnosis. The name "Creutzfeldt–Jakob disease" was introduced by Walther Spielmeyer in 1922, after the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob. CJD is caused by abnormal folding of a protein known as a prion. Infectious prions are misfolded proteins that can cause normally folded proteins to also become misfolded. About 85% of cases of CJD occur for unknown reasons, while about 7.5% of cases are inherited in an autosomal dominant manner. Exposure to brain or spinal tissue from an infected person may also result in spread. Ther ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Bovine Spongiform Encephalopathy
Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is an incurable and always fatal neurodegenerative disease of cattle. Symptoms include abnormal behavior, trouble walking, and weight loss. Later in the course of the disease, the cow becomes unable to function normally. There is conflicting information about the time between infection and onset of symptoms. In 2002, the World Health Organization suggested it to be approximately four to five years. Time from onset of symptoms to death is generally weeks to months. Spread to humans is believed to result in variant Creutzfeldt–Jakob disease (vCJD). , a total of 233 cases of vCJD had been reported globally. BSE is thought to be due to an infection by a misfolded protein, known as a prion. Cattle are believed to have been infected by being fed meat-and-bone meal that contained either the remains of cattle who spontaneously developed the disease or scrapie-infected sheep products. The United Kingdo ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Prion (bird)
The prions () or whalebirds are small petrels in the genera ''Pachyptila'' and '' Halobaena''. They form one of the four groups within the Procellariidae along with the gadfly petrels, shearwaters and fulmarine petrels. The name comes from the Greek ', meaning "saw", a reference of the serrated edges of the birds' saw-like bill. They are found in the Southern Ocean and breed on a number of subantarctic islands. Prions grow long, and have blue-grey upper parts and white underparts. Three species of prion have flattened bills with a fringe of lamellae that act as strainers for zooplankton.Maynard, B. J. (2003) All prions are marine and feed on small crustacea such as copepods, ostracods, decapods, and krill, as well as some fish such as myctophids and nototheniids. List of species * ''Pachyptila'' ** ''Pachyptila turtur'', fairy prion ** ''Pachyptila belcheri'', slender-billed prion ** ''Pachyptila crassirostris'', fulmar prion ** ''Pachyptila vittata'', broad-billed pri ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Chronic Wasting Disease
Chronic wasting disease (CWD), sometimes called zombie deer disease, is a transmissible spongiform encephalopathy (TSE) affecting deer. TSEs are a family of diseases thought to be caused by misfolded proteins called prions and include similar diseases such as BSE (mad cow disease) in cattle, Creutzfeldt–Jakob disease (CJD) in humans, and scrapie in sheep. Natural infection causing CWD affects members of the deer family. In the United States, CWD affects mule deer, white-tailed deer, red deer, sika deer, elk, bison, antelope, caribou, and moose. The transmission of CWD to other species such as squirrel monkeys and humanized mice has been observed in experimental settings. In 1967, CWD was first identified in mule deer at a government research facility in northern Colorado, United States. It was initially recognized as a clinical "wasting" syndrome and then in 1978, it was identified more specifically as a TSE disease. Since then, CWD has been found in free-rang ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Major Prion Protein
The major prion protein (PrP) is encoded in the human body by the ''PRNP'' gene also known as CD230 (cluster of differentiation 230). Expression of the protein is most predominant in the nervous system but occurs in many other tissues throughout the body. The protein can exist in multiple isoforms: the normal PrPC form, and the protease-resistant form designated PrPRes such as the disease-causing PrPSc (scrapie) and an isoform located in mitochondria. The misfolded version PrPSc is associated with a variety of cognitive disorders and neurodegenerative diseases such as in animals: ovine scrapie, bovine spongiform encephalopathy (BSE, mad cow disease), feline spongiform encephalopathy, transmissible mink encephalopathy (TME), exotic ungulate encephalopathy, chronic wasting disease (CWD) which affects deer; and in humans: Creutzfeldt–Jakob disease (CJD), fatal familial insomnia (FFI), Gerstmann–Sträussler–Scheinker syndrome (GSS), kuru, and variant Creutzfeldt–Jak ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Amyloids
Amyloids are aggregates of proteins characterised by a fibrillar morphology of typically 7–13 nm in diameter, a β-sheet secondary structure (known as cross-β) and ability to be stained by particular dyes, such as Congo red. In the human body, amyloids have been linked to the development of various diseases. Pathogenic amyloids form when previously healthy proteins lose their normal structure and physiological functions ( misfolding) and form fibrous deposits within and around cells. These protein misfolding and deposition processes disrupt the healthy function of tissues and organs. Such amyloids have been associated with (but not necessarily as the cause of) more than 50 human diseases, known as amyloidosis, and may play a role in some neurodegenerative diseases. Some of these diseases are mainly sporadic and only a few cases are familial. Others are only familial. Some result from medical treatment. Prions are an infectious form of amyloids that can act as a template ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Denaturation (biochemistry)
In biochemistry, denaturation is a process in which proteins or nucleic acids lose folded structure present in their native state due to various factors, including application of some external stress or compound, such as a strong acid or base, a concentrated inorganic salt, an organic solvent (e.g., alcohol or chloroform), agitation, radiation, or heat. If proteins in a living cell are denatured, this results in disruption of cell activity and possibly cell death. Protein denaturation is also a consequence of cell death. Denatured proteins can exhibit a wide range of characteristics, from conformational change and loss of solubility or dissociation of cofactors to aggregation due to the exposure of hydrophobic groups. The loss of solubility as a result of denaturation is called ''coagulation''. Denatured proteins, e.g., metalloenzymes, lose their 3D structure or metal cofactor, and therefore, cannot function. Proper protein folding is key to whether a globular or memb ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Alzheimer's Disease
Alzheimer's disease (AD) is a neurodegenerative disease and the cause of 60–70% of cases of dementia. The most common early symptom is difficulty in remembering recent events. As the disease advances, symptoms can include problems with language, disorientation (including easily getting lost), mood swings, loss of motivation, self-neglect, and behavioral issues. As a person's condition declines, they often withdraw from family and society. Gradually, bodily functions are lost, ultimately leading to death. Although the speed of progression can vary, the average life expectancy following diagnosis is three to twelve years. The causes of Alzheimer's disease remain poorly understood. There are many environmental and genetic risk factors associated with its development. The strongest genetic risk factor is from an allele of apolipoprotein E. Other risk factors include a history of head injury, clinical depression, and high blood pressure. The progression of the di ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Iatrogenesis
Iatrogenesis is the causation of a disease, a harmful complication, or other ill effect by any medical activity, including diagnosis, intervention, error, or negligence."Iatrogenic", ''Merriam-Webster.com'', Merriam-Webster, Inc., accessed 27 Jun 2020. First used in this sense in 1924, the term was introduced to sociology in 1976 by Ivan Illich, alleging that industrialized societies impair quality of life by overmedicalizing life."iatrogenesis" ''A Dictionary of Sociology'', . updated 31 May 2020. Iatrogenesis may thus include mental suffering via medical beliefs or a practitioner's statements. Some iatrogeni ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Stanley B
Stanley may refer to: Arts and entertainment Film and television * ''Stanley'' (1972 film), an American horror film * ''Stanley'' (1984 film), an Australian comedy * ''Stanley'' (1999 film), an animated short * ''Stanley'' (1956 TV series), an American situation comedy * ''Stanley'' (2001 TV series), an American animated series Other uses in arts and entertainment * ''Stanley'' (play), by Pam Gems, 1996 * Stanley Award, an Australian Cartoonists' Association award * '' Stanley: The Search for Dr. Livingston'', a video game Businesses * Stanley, Inc., an American information technology company * Stanley Aviation, an American aerospace company * Stanley Black & Decker, formerly The Stanley Works, an American hardware manufacturer ** Stanley Hand Tools, a division of Stanley Black & Decker * Stanley bottle, a brand of food and beverage containers * Stanley Electric, a Japanese manufacturer of electric lights * Stanley Furniture, an American furniture manufacturer * ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
