Creutzfeldt-Jakob disease and related transmissible spongiform encephalopathies

Article Abstract:

There is no clear cause of sporadic Creutzfeldt-Jakob disease despite intensive research. This is a neurologic disease that can ultimately lead to dementia. It belongs to a class of diseases called spongiform encephalopathies because they cause small holes in the brain, making it look like a sponge. They are also called prion diseases, because this brain damage is believed to be caused by a natural brain protein that somehow becomes abnormal and is then called a prion. Although bovine spongiform encephalopathy, or mad cow disease, may be transmitted through meat consumption, there is no evidence that Creutzfeldt-Jakob disease is transmitted in this way.

Health aspects, Prions, Prions (Proteins)

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Transmissible spongiform encephalopathies

Article Abstract:

Transmissible spongiform encephalopathies are fatal brain diseases in humans and animals that may be caused by newly-hypothesized infectious agents. They include scrapie, bovine spongiform encephalopathy, kuru, and Creutzfeldt-Jakob disease. The infectious agents may be small viruses, virinos, or prions, a replicating protein. Spongiform encephalopathies cause degeneration of brain tissue and changes in behavior. The bovine form, popularly called mad cow disease, may have resulted from feeding cows the remnants of infected animals. Transmission to humans may result from eating infected animals or brains.

Physiological aspects

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The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies

Article Abstract:

A new assay can detect the presence of a specific brain protein in cerebrospinal fluid that could be a marker for spongiform encephalopathies. This group of diseases is characterized by a degeneration of brain tissue and includes Creutzfeldt-Jakob disease in humans and mad cow disease. Two proteins discovered in cerebrospinal fluid have been found to be identical to the 14-3-3 brain protein. The immunoassay can detect the protein in cerebrospinal fluid of patients with Creutzfeldt-Jakob disease with a 95% accuracy. It also ruled out the disease in 96% of the patients with other types of dementia.

Diagnosis, Nerve proteins, Nerve tissue proteins, Cerebrospinal fluid

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