Human Creutzfeldt-Jakob Disease (CJD)

Creutzfeldt-Jakob Disease

85-94%

6%

10%

CJD

*neurodegenerative prion disease
"sporadic, genetic, infectious"

*misfolded prion protein isomer known as (PRP sc)

*Prion Protein (PRP) is coded by PRNP gene
*PRP is a transmembrance protein (alpha helices become beta sheets)
*beta sheets form amyloid fibrils then plaques
*caused by valine and glutamic acid transition to lysine

Reservoir: Humans, Mammals, Environment
Portal of entry: Digestive Tract, open wound
Mode of transmission: Infectious (contact with infected tissue or environmental piron), Kuru (ingestion of infected meat, this case human), Variant

Yes,
Hereditary/familiar (FCJD)

Makes up 10% of cases, onset 50-60 yrs old

variable (weeks-years)

*PRPsc (miss folded prion protein) resists digestion and induces misfolding of normal PRP prion proteins
*PRPsc forms water insoluble aggregates that damage nerve cells (impair brain function)

Dementia
Memory loss
Hallucinations
Paranoia
Obsessive-compulsive
Sleep disturbance
Problems with muscle control

*Severe brain damage
*Secondary infections
*Mortality - 85% with in two years of symptom onset. (Median life expectancy 4 months)

*don't eat contaminated animals
*avoid blood transfusions or tissue donations from those infected
*follow safety precautions
*incinerate contaminated material

*no cure
*palliative care

*humans, mammals, enviornment

digestive tract. open wounds

*sporadic (hereditary, familiar FCJD)
*infectious (ingestion of infected meat, contact with infected tissue or enviornmental prion)