Wednesday, December 3rd, 2008 384 views
Mutations in the PRNP gene cause prion disease.
Only a small percentage of prion disease cases run in families. Most cases are sporadic, which means they occur in people without any known risk factors or gene mutations. Rarely, prion diseases can be transmitted by accidental exposure to prion-contaminated tissues during a medical procedure. This type of prion disease is called iatrogenic.
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Prion Disease,
Rare Diseases
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Wednesday, December 3rd, 2008 396 views
Human Prion Diseases
- Creutzfeldt-Jakob Disease (CJD)
- Variant Creutzfeldt-Jakob Disease (vCJD)
- Gerstmann-Straussler-Scheinker Syndrome
- Fatal Familial Insomnia
- Kuru
Animal Prion Diseases
- Bovine Spongiform Encephalopathy (BSE)
- Chronic Wasting Disease (CWD)
- Scrapie
- Transmissible mink encephalopathy
- Feline spongiform encephalopathy
- Ungulate spongiform encephalopathy
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Prion Disease,
Rare Diseases
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Wednesday, December 3rd, 2008 331 views
Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.
The causative agent of TSEs is believed to be a prion. A prion is an abnormal, transmissible agent that is able to induce abnormal folding of normal cellular prion proteins in the brain, leading to brain damage and the characteristics signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.
Tags:
Prion Disease,
Rare Diseases
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