PRION DISEASES Meaning and
Definition
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Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of rare and fatal neurodegenerative disorders that primarily affect the brain and nervous system. These diseases are characterized by the accumulation of abnormally folded protein molecules, called prions, which lead to the progressive deterioration of brain tissue.
Prions are unique infectious agents as they are devoid of any nucleic acid material. The infectious prions can enter the body through ingestion, inhalation, or contact with contaminated tissues and can trigger the conversion of normal, harmless proteins into an abnormal conformation. This conformational change causes the accumulation and aggregation of abnormal prions, eventually leading to the formation of amyloid plaques within the brain.
The symptoms of prion diseases typically include cognitive decline, memory loss, personality changes, impaired coordination, and involuntary movements. As the disease progresses, individuals experience severe neurological dysfunction, including dementia, muscle stiffness, and difficulties in speech and swallowing. Prion diseases are invariably fatal, with a rapidly progressive and irreversible course.
There are several types of prion diseases that affect both humans and animals, including Creutzfeldt-Jakob disease (CJD), variant CJD (vCJD), kuru, Gerstmann-Sträussler-Scheinker syndrome (GSS), and fatal familial insomnia (FFI). While some prion diseases are sporadic and occur spontaneously, others can be inherited through genetic mutations or acquired through exposure to infected tissues. Given the highly infectious and fatal nature of prion diseases, understanding their transmission, diagnosis, and potential treatments remains an ongoing area of scientific research and medical investigation.
Common Misspellings for PRION DISEASES
- orion diseases
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Etymology of PRION DISEASES
The term "prion diseases" was first introduced in the late 1980s to describe a group of transmissible neurodegenerative diseases. The word "prion" itself is a combination of protein and infectious, as these diseases are characterized by the accumulation of abnormal, misfolded proteins in the brain.
The word "prion" was coined by Dr. Stanley B. Prusiner, an American neurologist, in reference to the infectious agent responsible for these diseases. Prusiner received the Nobel Prize in Physiology or Medicine in 1997 for his discovery of prions and their role in causing diseases like Creutzfeldt-Jakob disease and variant Creutzfeldt-Jakob disease.
The term "prion" originated from the words "proteinaceous" and "infection". It signifies that the infectious agent causing these diseases is not a virus or a bacterium but rather an abnormally folded protein.
Similar spelling words for PRION DISEASES
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