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Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, [1] 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.
A prion / ˈ p r iː ɒ n / ⓘ 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.
Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is an incurable and invariably fatal neurodegenerative disease of cattle. [2] Symptoms include abnormal behavior, trouble walking, and weight loss. [1] Later in the course of the disease, the cow becomes unable to function normally. [1]
A downed animal suffering from Bovine spongiform encephalopathy A downer is an animal, usually livestock , that cannot stand on its own and therefore is to be killed. A downed animal, one that is unable to stand, is not necessarily a downer.
Transmissible spongiform encephalopathy: A collection of diseases all caused by prions, and characterized by "spongy" brain tissue (riddled with holes), impaired locomotion or coordination, and a 100% mortality rate. Includes bovine spongiform encephalopathy (mad cow disease), scrapie, and kuru among others.
Creutzfeldt–Jakob disease (CJD), also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is a fatal neurodegenerative disease. [ 4 ] [ 1 ] Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. [ 4 ]
Kuru, a transmissible spongiform encephalopathy, is a disease of the nervous system that causes physiological and neurological effects which ultimately lead to death. It is characterized by progressive cerebellar ataxia , or loss of coordination and control over muscle movements.
Spongiform cardiomyopathy: Noncompaction cardiomyopathy is inherited in an autosomal dominant manner: Specialty: Cardiology ...