Materiały źródłowe

• #1 Creutzfeldt-Jakob disease – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/creutzfeldt-jakob-disease/symptoms-causes/syc-20371226

  Creutzfeldt-Jakob disease and related conditions appear to be caused by changes to a type of protein called a prion. These proteins are typically produced in the body. But when they encounter infectious prions, they fold and become another shape that’s not typical. They can spread and affect processes in the body. […] Prions are proteins that occur naturally in the brains of animals and people. Normally, the proteins are harmless, but when they’re misshapen, they can cause devastating illnesses such as disease in cattle and Creutzfeldt-Jakob disease in humans. […] The risk of getting CJD is low. The disease can’t be spread through coughing or sneezing. It also can’t be spread by touching or sexual contact. CJD can develop in three ways: […] Most people with Creutzfeldt-Jakob disease develop the disease for no apparent reason. This type, called spontaneous CJD or sporadic CJD, accounts for most cases.

• #2 Creutzfeldt-Jakob Disease | National Institute of Neurological Disorders and Stroke

  https://www.ninds.nih.gov/health-information/disorders/creutzfeldt-jakob-disease

  Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressing brain disorder. […] CJD and other prion diseases are caused by abnormal forms of proteins called prions. […] Sporadic CJD and other prion diseases may develop because some of a person’s normal prion proteins suddenly change into abnormal prions. […] Gene mutations that affect normal prion protein production can run in families. […] CJD and other TSEs cant transmit through the air or through touching or most other forms of casual contact. […] A type of CJD called variant CJD (or vCJD) comes from eating meat from cattle affected by a prion disease called bovine spongiform encephalopathy (BSE, or mad cow disease). […] Researchers are examining and characterizing the prions associated with CJD and other human and animal prion diseases. […] Researchers are exploring genetic regulation of the prion protein gene to change the course of prion disease and hopefully significantly slow it or stop it altogether.

• #3 Creutzfeldt-Jakob Disease (CJD): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/6001-creutzfeldt-jakob-disease

  Creutzfeldt-Jakob disease (CJD) is a severe, degenerative brain condition. It happens when faulty proteins, known as prions, damage your brain. This condition usually worsens very quickly, and most people dont survive more than a year after diagnosis. Most cases of CJD happen for unknown reasons, but 10% to 15% of cases are inherited. […] With CJD, faulty proteins, known as prions, build up in your brain cells, damaging and destroying those cells. The condition is very severe, and its effects develop and worsen quickly. Its ultimately fatal, and unfortunately, theres no way to cure, treat or even slow down the progress of this disease. […] Faulty proteins in your brain known as prions cause CJD. Proteins are chemical molecules that need to hold a specific shape to work (like how a lock opens for a key with the right shape).

• #4

  https://www.nhs.uk/conditions/creutzfeldt-jakob-disease-cjd/

  Creutzfeldt-Jakob disease (CJD) appears to be caused by an abnormal infectious protein called a prion. These prions accumulate at high levels in the brain and cause irreversible damage to nerve cells. […] The precise cause of sporadic CJD is unclear, but it’s been suggested that a normal brain protein changes abnormally („misfolds”) and turns into a prion. […] Variant CJD (vCJD) is likely to be caused by consuming meat from a cow that had bovine spongiform encephalopathy (BSE, or „mad cow” disease), a similar prion disease to CJD. […] Familial CJD is a very rare genetic condition where one of the genes a person inherits from their parent (the prion protein gene) carries a mutation that causes prions to form in their brain during adulthood, triggering the symptoms of CJD. […] Iatrogenic CJD is where the infection is accidentally spread from someone with CJD through medical or surgical treatment.

• #5 Creutzfeldt-Jakob Disease (CJD): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/6001-creutzfeldt-jakob-disease

  When your body makes faulty proteins for any reason, those misshapen proteins can cause degenerative brain diseases, like Alzheimers disease. […] But prion-based diseases have a key difference. Instead of a slow build-up of faulty proteins, prions convert normal proteins into more prions. As the number of prions grows, they turn more proteins into prions. The more prions there are, the faster the conversion happens. […] Prions are also dangerous because theyre more resilient than most microbes. Cooking temperatures dont destroy prions like viruses or bacteria. Your immune system cant stop prions, so theres no way to develop an immunity to CJD or a vaccine to prevent it. […] The different reasons are what determine the type of CJD you have. The different types are: Sporadic CJD. This is the most common kind of CJD, making up about 85% to 90% of cases. It happens for unknown reasons. Genetic CJD. This type happens because of a genetic mutation you inherit from one or both parents. It ranks second among the most common causes, making up between 10% and 15% of CJD cases. Acquired CJD. This is CJD you acquire from various sources, such as medical procedures like organ or tissue transplants and grafts, contaminated surgical equipment, etc. Variant CJD (vCJD). This happens after eating beef from a cow with bovine spongiform encephalopathy (BSE), another disease that happens because of prions.

• #6 Creutzfeldt-Jakob disease (CJD): Symptoms, causes, and treatment

  https://www.medicalnewstoday.com/articles/185884

  Creutzfeldt-Jakob disease (CJD) is caused by a prion, a misfolded protein that can transmit its malformation to healthy variants of the same protein. […] Scientists believe that prions are responsible for CJD and other TSEs. […] A prion is a type of protein with an abnormal structure, and it passes this abnormality on to other proteins. This damages brain tissue and causes the characteristic symptoms of CJD. […] CJD may be inherited or acquired. In some cases, it develops sporadically, without an identifiable pattern. […] In 85% of cases, CJD is sporadic. This means that there is no clear reason why it develops. […] A person may have a family history of CJD. Between 10% and 15% of CJD cases are inherited. […] The disease can develop if a change occurs in the gene that controls the formation of prion proteins. […] Fewer than 1% of CJD cases are acquired. […] It is possible to acquire vCJD, not classic CJD, by eating meat from a cow that had bovine spongiform encephalopathy. A person cannot acquire classic CJD through food.

• #7 Creutzfeldt-Jakob Disease: Symptoms, Causes, and CJD in Australia

  https://brainfoundation.org.au/disorders/creutzfeldt-jakob-disease/

  Variant CJD (vCJD) was first reported in 1996 following the first death of an affected individual in 1994 in the UK. Globally, there have been 232 cases, mainly in the UK, with the vast majority occurring as a result of the consumption of contaminated meat products from bovine spongiform encephalopathy (BSE) affected cattle. […] CJD is always fatal and is unfortunately distinctive because of its typically rapid clinical progression.

• #8 Creutzfeldt-Jakob disease Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/creutzfeldt-jakob-disease

  Creutzfeldt-Jakob disease (CJD) is a form of brain damage that leads to a rapid decrease in movement and loss of mental function. […] CJD is caused by a protein called a prion. A prion causes normal proteins to fold abnormally. This affects other proteins’ ability to function. […] Sporadic CJD makes up most cases. It occurs for no known reason. It starts on average at age 65 years. […] Familial CJD occurs when a person inherits the abnormal prion from a parent (this form of CJD is rare). […] Acquired CJD includes variant CJD (vCJD), the form related to mad cow disease. […] Variant CJD is caused by eating infected meat. The infection that causes the disease in cows is thought to be the same one that causes vCJD in humans. […] Iatrogenic CJD is also an acquired form of the disease. Iatrogenic CJD is sometimes passed through a blood product transfusion, transplant, or contaminated surgical instruments.

• #9 Creutzfeldt-Jakob disease (CJD) | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/creutzfeldt-jakob-disease-cjd

  CJD is extremely rare. It affects at least one person in every million people, within the total population each year. […] In almost 90 per cent of cases, it is not understood how or why CJD occurs. […] The cause of sporadic CJD is unknown. […] It is not known what causes the initial change to the prion protein structure. It is thought it could be triggered by: Changes in the brains chemical environment, Exposure to abnormal prion protein for example, in medical exposures described previously, Genetic influences that may cause some people to be more vulnerable to spontaneously producing the abnormal prion protein. […] A viral connection remains unproven.

• #10 Creutzfeldt-Jakob disease (CJD) | Britannica

  https://www.britannica.com/science/Creutzfeldt-Jakob-disease

  Creutzfeldt-Jakob disease (CJD), rare fatal degenerative disease of the central nervous system. CJD occurs throughout the world at an incidence of one in every one million people. […] CJD, as well as other spongiform encephalopathies, is caused by an unusual pathogenic agent called a prion. A prion is a deviant form of a normally harmless protein found in the brains of mammals and birds. As prions replicate by converting normal forms of the protein into their abnormal shape they accumulate within nerve cells, causing neurodegeneration. […] Acquired CJD, which occurs through infection with the prion protein, accounts for only 1 percent of all CJD cases. Sporadic (i.e., occurring at random) forms account for the majority of cases at least 85 percent. In these cases it is unclear what molecular process causes the prion protein to appear in the first place. The protein may arise from a mutation incurred as the body ages or as a result of a spontaneous conversion in the proteins shape.

• #11 Creutzfeldt-Jakob disease | Handouts | MedLink Neurology

  https://www.medlink.com/handouts/creutzfeldt-jakob-disease

  Creutzfeldt-Jakob disease is caused by abnormal forms of proteins called prions. […] The infectious prions are believed to be made by an error in the cell’s machinery that makes proteins and controls their quality. These errors are more likely to occur with aging. […] In the acquired form of the disease, the infectious PrPSc comes from the outside the body, for example, through contaminated meat as is seen in variant Creutzfeldt-Jakob disease. […] In the hereditary form, infectious prions can arise when a mutation occurs in the gene for the body’s normal prion protein. […] In the sporadic form, the infectious prions are believed to be made by an error in the cell’s machinery that makes proteins and controls their quality.

• #12 Creutzfeldt-Jakob disease – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/creutzfeldt-jakob-disease/symptoms-causes/syc-20371226

  Fewer than 15% of people with CJD have a family history. They may test positive for genetic changes associated with the disease. This type is referred to as familial CJD. […] A small number of people have developed CJD as a result of medical procedures. These procedures included injections of pituitary human growth hormone from an infected source. […] A small number of people have developed variant CJD from eating contaminated beef. Variant CJD is linked to eating beef from cattle infected with mad cow disease. Mad cow disease is known as bovine spongiform encephalopathy (BSE).

• #13

  https://www.nhs.uk/conditions/creutzfeldt-jakob-disease-cjd/causes/

  Creutzfeldt-Jakob disease (CJD) is caused by an abnormal infectious protein in the brain called a prion. […] The different types of CJD are all caused by a build-up of prions in the brain. But the reason why this happens is different for each type. […] There’s clear evidence that variant CJD (vCJD) is caused by the same strain of prions that causes bovine spongiform encephalopathy (BSE, or „mad cow” disease). […] Familial or inherited CJD is a rare form of CJD caused by an inherited mutation (abnormality) in the gene that produces the prion protein. […] Iatrogenic CJD (iCJD) is where the infection is spread from someone with CJD through medical or surgical treatment. […] One theory about why BSE developed is that an older prion disease that affects sheep, called scrapie, may have mutated.

• #14 Creutzfeldt-Jakob disease (CJD) | Britannica

  https://www.britannica.com/science/Creutzfeldt-Jakob-disease

  Between 5 and 15 percent of CJD cases show a familial pattern of inheritance. In these inherited cases a mutation in a gene designated PRNP), which encodes the prion protein PrP, is passed from parent to child in a dominant fashion (i.e., only one of the two copies of the gene that are inherited one from each parent need be mutated for disease to occur). More than 50 different mutations in PRNP have been identified. While some of these mutations cause CJD, others cause Gerstmann-Strussler-Scheinker syndrome and fatal familial insomnia. In addition, mutations have been identified that do not cause disease but may render individuals more susceptible to infection with the prion. These latter mutations may be involved in some of the sporadic incidences of the disease.

• #15 Creutzfeldt-Jakob Disease (CJD) | Symptoms & Treatments | alz.org

  https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/creutzfeldt-jakob-disease

  Creutzfeldt-Jakob disease (CJD) is the most common human form of a group of rare, fatal brain disorders known as prion diseases. […] Prion diseases, such as Creutzfeldt-Jakob disease, occur when prion protein, which is found throughout the body but whose normal function isn’t yet known, begins folding into an abnormal three-dimensional shape. […] Sporadic CJD has no known cause. Most scientists believe the disease begins when prion protein somewhere in the brain spontaneously misfolds, triggering a „domino effect” that misfolds prion protein throughout the brain. […] Familial CJD is caused by variations in the prion protein gene that increase the likelihood an individual will develop CJD. […] Researchers have identified more than 50 prion protein mutations in those with inherited CJD.

• #16

  https://www.healthshare.com.au/questions/43367-what-causes-creutzfeldt-jakob-disease/

  This answers depends on which form of Creutzfeldt-Jakob disease the question is referring to. Sporadic CJD – It is believed to be the result of a spontaneous conformational change in the native prion protein that occurs at random in about 1 to 2 people per million of the population per year. […] Genetic CJD – Is an inherited forms of prion disease. The form of CJD is caused by a mutation in the prion protein gene that is typically inherited in families in an autosomal dominant fashion, meaning that from generation to generation approximately 50% of family members will carry the mutation and develop CJD during their lifetime if they live long enough. […] Acquired forms of CJD are caused by a transmission incident that has exposed the patient to contaminated prions. […] Iatrogenic or medically acquired CJD – exposure to contaminated surgical instruments through an invasive medical procedure or contaminated medical products ie human pituitary hormones of dura mater grafts. […] Kuru – transmitted through cannibalism. […] Variant CJD, commonly known as Mad Cow disease is recognised to be caused by the consumption of contaminated BSE beef (No identified cases in Australia).

• #17 Creutzfeldt-Jakob Disease (CJD): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/6001-creutzfeldt-jakob-disease

  The causes of CJD are as follows: Sporadic (unknown cause): 85% and 90% of cases. Genetic CJD (conditions inherited from one or both parents): 10% to 15% of cases. Acquired CJD (infection from a medical procedure, such as a transplant or tissue graft): Less than 1% of cases. Variant CJD (contracted by eating beef from an animal with bovine spongiform encephalopathy): Only 232 confirmed cases since 1986. […] No, CJD is always fatal. Thats because your immune system cant recognize the problem and cant fight back against it as it can with other infectious diseases.

• #18 About Variant Creutzfeldt-Jakob Disease (vCJD) | vCJD | CDC

  https://www.cdc.gov/variant-creutzfeldt-jakob/about/index.html

  Variant Creutzfeldt-Jakob disease (vCJD) is a rare disease caused by a prion, an infectious protein. […] It is linked to eating beef from cows infected with „mad cow disease.” […] There is strong evidence vCJD is caused when people eat meat from cows that were infected with Bovine Spongiform Encephalopathy (BSE). […] Scientists believe people may have eaten food potentially contaminated with BSE in the mid-1980s. vCJD cases started appearing about 10 years later. This matches the usual incubation period (the time between exposure and symptoms) for other prion diseases.

• #19 Creutzfeldt-Jakob Disease

  https://dph.illinois.gov/topics-services/diseases-and-conditions/diseases-a-z-list/diseases/creutzfeldt-jakob-disease.html

  Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain disorder caused by a prion. […] There are various forms of CJD; in about 85 percent of the cases, the cause is unknown. One form of CJD may be linked to consumption of beef in Great Britain. […] Scientists agree that a prion that is transmissible causes Creutzfeldt-Jakob disease, but this agent has not yet been fully identified. […] A prion is a protein comprising a specific sequence of amino acids. It is neither a virus nor any other previously known infectious agent, but rather an unconventional agent. Prions are thought to transform normal, benign protein molecules into deadly ones by altering their shape. […] In 1996, officials in Great Britain announced that 10 cases of a previously unrecognized form of CJD had been identified and might be linked to consumption of beef from cattle with bovine spongiform encephalopathy (BSE), a disease that affected more than 177,500 cattle in that country from 1986 through 2000.

• #20 Creutzfeldt-Jakob disease Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/creutzfeldt-jakob-disease

  CJD may be related to several other diseases caused by prions, including: Chronic wasting disease (found in deer), Kuru (affected mostly women in New Guinea who ate the brains of dead relatives as part of a funeral ritual), Scrapie (found in sheep), Other very rare inherited human diseases, such as Gerstmann-Straussler-Scheinker disease and fatal familial insomnia.

• #21 Creutzfeldt-Jakob disease | Lima Memorial Health System

  https://www.limamemorial.org/m/health-library/HIE%20Multimedia-TextOnly/1/000788

  CJD may be related to several other diseases caused by prions, including: Chronic wasting disease (found in deer), Kuru (affected mostly women in New Guinea who ate the brains of dead relatives as part of a funeral ritual), Scrapie (found in sheep), Other very rare inherited human diseases, such as Gerstmann-Straussler-Scheinker disease and fatal familial insomnia.

• #22 Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: Background, Pathophysiology and Etiology, Epidemiology

  https://emedicine.medscape.com/article/1169688-overview

  Bovine spongiform encephalopathy (BSE), also known as mad cow disease, and variant Creutzfeldt-Jakob disease (CJD) are related disorders. They belong to the family of diseases known as the transmissible spongiform encephalopathies (TSEs). TSEs are caused by a transmissible proteinaceous particle, which is yet to be fully characterized. […] Epidemiologic, biological, and biochemical data favor the hypothesis that variant CJD is a BSE zoonosis, probably arising from a double-species switch from sheep scrapie to BSE and then from bovine spongiform encephalopathy (BSE) to human variant CJD. […] The most compelling hypothesis is that BSE originated from scrapie, an endemic spongiform encephalopathy of sheep and goats that has been endemic in Europe since the mid-18th century. […] The causative agent is suspected to be from either scrapie-affected sheep or cattle with previously unidentified TSE.

• #23 Prion Diseases | Brain Institute | OHSU

  https://www.ohsu.edu/brain-institute/prion-diseases

  Creutzfeldt-Jakob disease has several forms: Sporadic CJD develops spontaneously for no known reason, typically in people ages 60 to 65. Familial CJD affects people who inherited a gene mutation from a parent. It accounts for about 10 to 15 percent of cases. Some genetic types appear in people ages 20 to 40. Acquired CJD, including Variant Creutzfeldt-Jakob disease, or vCJD, results from exposure to an outside source of abnormal prion protein. […] Other seldom-seen forms of prion disease are: Variably protease-sensitive prionopathy, similar to CJD but more likely to strike people around age 70 who have a family history of dementia. Familial fatal insomnia, which affects the area of the brain that controls sleep. Kuru, spread by cannibalism or other contact with the tissue of infected people. Gerstmann-Straussler-Scheinker syndrome, a progressive brain disorder that is almost always inherited.

• #24 Creutzfeldt-Jakob Disease and Mad Cow Disease

  https://www.healthline.com/health/creutzfeldt-jakob-disease

  Theres strong evidence that the agent responsible for bovine spongiform encephalopathy (BSE) in cows, commonly called mad cow disease, is also responsible for one form of acquired CJD in humans, called variant CJD. […] Variant CJD (vCJD) first appeared in humans in 1994-1996, about a decade after people first had extended exposure to potentially BSE-contaminated beef.

• #25 Creutzfeldt-Jakob Disease

  https://www.health.ny.gov/diseases/communicable/creutzfeldt-jakob/fact_sheet.htm

  Creutzfeldt-Jakob disease, often referred to as CJD, is a rare, fatal disease affecting the nervous system. […] For 80 to 90 percent of the people diagnosed with CJD, scientists do not know the cause. These individuals are referred to as having sporadic CJD. Between 5 percent and 15 percent of CJD cases are genetic, meaning the risk for the disease is inherited. These individuals are referred to as having familial CJD. Finally, in less than 1 percent of all cases, CJD is caused by direct exposure to tissue contaminated with CJD through specific types of medical procedures involving nervous system tissue. […] The cause of CJD is believed to be a prion, an abnormal protein that can occur in certain types of nervous system tissue. CJD is not caused by a bacteria, virus, or parasite. […] Most cases of CJD (sporadic, familial) are not caused by or related to eating beef. Variant CJD (vCJD), more commonly known as „mad cow disease,” was first described in 1996 in the United Kingdom and was linked to eating beef from cattle infected with the animal form of the disease (BSE bovine spongiform encephalopathy). There is strong scientific evidence that the agent responsible for the outbreak of prion disease in cows is the same agent responsible for the outbreak of vCJD in humans.

• #26 Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: Background, Pathophysiology and Etiology, Epidemiology

  https://emedicine.medscape.com/article/1169688-overview

  Changes in the rendering process that took place in the early 1980s, particularly the removal of a solvent extraction process that included a steam heat treatment, probably allowed the etiologic agent to survive, contaminate the protein supplement, and infect cattle. […] Recognition of the source of infection led to several countermeasures to break the cycle of cattle reinfection, restrict the geographic spread, and eliminate the potential source of new infection. […] Convincing evidence indicates that variant Creutzfeldt-Jakob disease (CJD) is a new disease. […] The amount of infectious agent ingested and host susceptibility, as determined by the human genotype at PRNP codon 129, appear to play important roles in the development of variant CJD.

• #27 Creutzfeldt Jakob Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK507860/

  Creutzfeldt-Jakob disease (CJD) is a rare, fatal degenerative brain disorder caused by prion proteins. […] CJD may be classified based on the mode of transmission. Sporadic CJD, the most common type (85%), is due to misfolding of normal PrP isoforms with no apparent triggers. […] Genetic CJD, the second most common type (10-15%), arises from a heritable genetic mutation. […] Infectious CJD accounts for less than 1% of cases, and it arises from prion transmission by an external source. […] Prions reproduce by associating with normal PrP cellular isoforms, converting -helices into indigestible -pleated sheets. These particles cause CJD and other transmissible spongiform encephalopathies like bovine spongiform encephalopathy (mad cow disease), kuru, and scrapie. […] CJD primarily affects the central nervous system (CNS). […] The condition has a long incubation period. […] CJD neuronal inclusions damage brain neurons, manifesting with nonspecific prodromal symptoms early in the disease course and neurologic changes, particularly myoclonus, in the advanced stages.

• #28 Creutzfeldt–Jakob disease – Wikipedia

  https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease

  CreutzfeldtJakob disease (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. […] CJD is a type of transmissible spongiform encephalopathy (TSE), which are caused by prions. […] The CJD prion is dangerous because it promotes refolding of the cellular prion protein into the diseased state. […] Mutations in the gene for the prion protein can cause a misfolding of the dominantly alpha helical regions into beta pleated sheets. […] Once the prion is transmitted, the defective proteins invade the brain and induce other prion protein molecules to misfold in a self-sustaining feedback loop. […] It is thought that humans can contract the variant form of the disease by eating food from animals infected with bovine spongiform encephalopathy (BSE), the bovine form of TSE, also known as mad cow disease. […] All types of CJD are transmissible irrespective of how they occur in the person.

• #29 Creutzfeldt-Jakob disease – Causes, Symptoms, Treatment, Diagnosis – MedBroadcast.com

  https://medbroadcast.com/condition/getcondition/creutzfeldt-jakob-disease

  There was a problem in the theory, however. No one had found a bacterium or virus that caused any of these diseases. In fact, scrapie-infected tissue was still known to be infectious even after being exposed to radiation that would destroy DNA, and thus kill any bacterium or virus. Finally, it was discovered that a protein called a prion seemed to be causing the damage. […] Prions tend to accumulate in nerve cells, and cuts of meat that come from near the spinal cord and brain are the most dangerous. You are probably less likely to eat prions if you eat cuts of meat from other parts of the animal (e.g., steaks, roasts). Keep in mind that prions are not destroyed by cooking meat.

• #30 Creutzfeldt-Jakob Disease and Mad Cow Disease

  https://www.healthline.com/health/creutzfeldt-jakob-disease

  Creutzfeldt-Jakob Disease (CJD) is an infectious disease that causes the brain to degenerate. […] CJD occurs due to abnormal changes to cellular proteins called prions. […] When you develop sporadic or inherited CJD, one of the proteins responsible for creating prions becomes abnormal and produces an infected prion. […] The infected prion then spread through the central nervous system in a chain reaction or like dominoes getting knocked over until it eventually reaches the brain and causes the development of lesions or holes. […] An error within your cells creates the infected prion. This is more common in advanced age. […] A change in your genetic code creates an infected prion, which you can then pass on to your children through sperm or egg cells. […] In acquired forms, including variant CJD, the prion comes from outside of the body, often in the form of eating infected meat.

• #31 Creutzfeldt-Jakob Disease Causes and Prognosis

  https://www.verywellhealth.com/creutzfeldt-jakob-disease-5410212

  Once the damaged prions are introduced, they cause the development of other prions that fold up incorrectly in the brain. […] CJD is believed to cause rapidly progressive changes in the brain. […] The disease can occur without a known cause or trigger. It can also run in families or be acquired through contact with contaminated tissue.

• #32 Creutzfeldt-Jakob Disease (CJD) | Symptoms & Treatments | alz.org

  https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/creutzfeldt-jakob-disease

  Variation at codon 129 in the prion protein gene may also play a role in making people susceptible to acquired CJD from external sources. […] Scientists dont yet know why acquired CJD seems to be transmitted through such a limited number of external sources. […] The two most common outside sources are: Medical procedures involving instruments used in neurosurgery, growth hormone from human sources or certain transplanted human tissues, including corneas (the clear outer covering of the eye) and dura mater (the fibrous membrane covering the brain and spinal cord). […] Meat or other products from cattle infected with bovine spongiform encephalopathy (BVE) or „mad cow disease,” recognized in the mid-1990s as the cause of variant CJD (vCJD).

• #33 Creutzfeldt-Jakob Disease (CJD) – Brain, Spinal Cord, and Nerve Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/brain-spinal-cord-and-nerve-disorders/prion-diseases/creutzfeldt-jakob-disease-cjd

  Creutzfeldt-Jakob disease usually occurs spontaneously but may result from eating contaminated beef or from inheriting an abnormal gene. […] Creutzfeldt-Jakob disease (CJD) is a prion disease, which develops when a normal protein called cellular prion protein (PrPC) changes shape (misfolds) and becomes a disease-causing prion. […] Familial CJD results from a mutation in the gene for PrPC, which causes normal PrPC to change into disease-causing prion. […] Acquired CJD can result from eating contaminated beef (called variant CJD) […] CJD can also be acquired during a medical procedure (called iatrogenic CJD). […] Iatrogenic CJD has been acquired when hormones derived from human pituitary glands were used for treatment. […] Only 3 people acquired vCJD from transfusion of contaminated blood and developed symptoms. […] Because there is no effective treatment for Creutzfeldt-Jakob disease, preventing its spread is essential. Only acquired CJD can be prevented.

• #34 Creutzfeldt-Jakob Disease (CJD) – Harvard Health

  https://www.health.harvard.edu/a_to_z/creutzfeldt-jakob-disease-cjd-a-to-z

  It is extremely uncommon for CJD to spread from one person to another. However, in very rare cases CJD has been transmitted by a blood transfusion, by a medical procedure (because of contaminated equipment), by contaminated tissue (such as corneas that are used for transplant) or by injections of hormones extracted from human tissues.

• #35 Mad Cow Disease: Symptoms, Causes and Treatments for vCJD

  https://www.webmd.com/brain/mad-cow-disease-basics

  Mad cow disease or bovine spongiform encephalopathy (BSE) can cause a rare but fatal disease in people called variant Creutzfeldt-Jakob Disease (vCJD). […] Instead of viruses or bacteria, it’s caused by an infection from an abnormal version of a protein, called a prion. For reasons still unknown, this protein becomes harmful and destroys nervous system tissue in the brain and spinal cord. […] There’s strong evidence people can get vCJD by eating beef products with central nervous system tissue, such as brain and spinal cord, from cattle infected with mad cow disease. […] Classic CJD has no known cause and occurs each year at a rate of one to two cases per million people throughout the world, including the U.S. and countries where mad cow disease has never been reported. […] The best way to prevent vCJD is to prevent cows from getting BSE. […] The human version, called vCJD, can spread when people eat beef contaminated with brain or spinal cord tissue from an infected cow, or if they receive blood from an infected person.

• #36 Creutzfeldt-Jakob disease: a rapidly progressing form of dementia — DPUK

  https://www.dementiasplatform.uk/news-and-media/blog/creutzfeldt-jakob-disease-a-rapidly-progressing-form-of-dementia

  There are three main subtypes of prion disease, the main one being sporadic CJD, which affects 85% of people with the condition. […] The second most common type is inherited prion disease, which causes roughly 10-15% of cases. […] The final subtype is acquired CJD, the category which variant CJD the form linked with BSE falls into. […] Acquired CJD is contracted from exposure to prion protein from an external source, such as through eating infected produce or receiving medical procedures where human tissues are taken from cadavers (dead bodies used in medicine). […] Cases of acquired CJD have fallen dramatically due to greater awareness of the condition, improved animal feeding and halting the use of cadaveric medical and surgical products.

• #37 CEUFast – Creutzfeldt-Jakob Disease (CJD)

  https://ceufast.com/course/creutzfeldt-jakob-disease-cjd

  Several different mutations in the prion gene have been identified. […] The specific mutation found in each family affects how frequently the disease appears and what symptoms are most noticeable. […] Laboratory tests have shown a strong similarity between the prions causing BSE and v-CJD. […] Researchers are studying CJD and are examining whether the transmissible agent is a prion or a product of the infection.

• #38 Creutzfeldt-Jakob Disease: Causes, Symptoms, And Treatment | The Lifesciences Magazine

  https://thelifesciencesmagazine.com/explore-creutzfeldt-jakob-disease-cjd/

  While most cases of CJD are sporadic, some are familial and linked to mutations in the PRNP gene. However, familial CJD is relatively rare compared to the sporadic form. […] Iatrogenic CJD can occur through medical procedures involving contaminated tissues or instruments. Variant CJD, associated with consumption of contaminated beef products, is a concern, although strict food safety measures have reduced its incidence.

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