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Creutzfeldt-Jakob Disease (CJD)

Organism, Causative Agent, or Etiologic Agent

Prion (infectious protein)

Description

Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of progressive neurodegenerative disorders in humans and animals. The causative agents of TSEs are believed to be prions. Prion proteins are normal cellular proteins that are found mostly in the brain. The function of these proteins are still not completely understood. The term “prions” refers to abnormal, pathogenic prion proteins that are transmissible. They are able to induce abnormal folding of normal cellular prion proteins. As the amount of abnormal prion protein grows, it becomes hard to break down, causing brain degeneration and neurologic disease. Prion diseases are always fatal and have long incubation periods that are often measured in years. Creutzfeldt-Jakob disease (CJD) is the most common human prion disease. Sporadic CJD makes up 85-95% of all CJD cases, followed by genetic or familial CJD with 5-15% of cases; <1% of cases are iatrogenic or variant CJD.

General Information and Resources

Mailing Address

Texas Department of State Health Services
Emerging and Acute Infectious Disease Unit
P.O. Box 149347, Mail Code: 1960
Austin , TX 78714-9347
United States

Physical Address

Emerging and Acute Infectious Disease Unit
Moreton Building, Suite M-631, Mail Code: 1960
1100 West 49th Street
Austin , TX 78756-3199
United States