Anatomy and Cell Biology 4451F/G Lecture Notes - Lecture 19: Transmissible Spongiform Encephalopathy, Fatal Familial Insomnia, Kuru (Disease)

• School: Western University
• Department: Anatomy and Cell Biology
• Course: Anatomy and Cell Biology 4451F/G
• Professor: Suzanne Schmid

Document Summary

Lecture 19 flavio beraldo prions diseases and prion protein signaling. Affect the brain causing neurodegeneration (dementia (cognitive impairment) and motor impairment) Long asymptomatic period of incubation hard to diagnose. Two human prion diseases (kuru and classic cjd?) You get holes in the brain, looks like a sponge (hence name) Fse feline spongiform encephalopathy (cats, lions etc) So when first case discovered, all boarders were closed: west (cid:449)ould(cid:374)"t (cid:271)u(cid:455) (cid:373)eat a(cid:374)(cid:455)(cid:373)ore. Increase on import not even canadians wanted to consume canadian beef. Leads to death within 1 year of onset (once symptoms show, you die in a year) Long period of incubation (could last 40 years) Cerebrospinal fluid csf (specific proteins linked to tse) not 100% reliable. Behavioral (dementia: combine these 3 to make sure someone has disease or not. Rate of 1. 5 cases per million per year (400 new cases in the us per year: numbers may be way higher.