Handout 9 - Prions

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Humans are also susceptible to several prion diseases: •The incidence of sporadic CJD is about 1 per million per year. •GSS occurs at about 2% of the rate of CJD. •It is estimated that 1 in 10,000 people are infected with CJD at the time of death. These figures are likely to be underestimates since prion diseases may be misdiagnosed as other neurological disorders. 3/26/2006 Humans are also susceptible to several prion diseases: •The diseases are characterized by loss of motor control, dementia, paralysis wasting and eventually death, typically following pneumonia. • Fatal Familial Insomnia presents with an untreatable insomnia and dysautonomia. Details of pathogenesis are largely unknown. •Visible end results at post-mortem are non-inflammatory lesions, vacuoles, amyloid protein deposits and astrogliosis. 3/26/2006
Humans are also susceptible to several prion diseases: •GSS is distinct from CJD, it occurs typically in the 4th-5th decade, characterized by cerebellar ataxia and concomitant motor problems, and less common dementia. •(Originally thought to be familial, but now known to occur sporadically as well). 3/26/2006 Humans are also susceptible to several prion diseases: •CJD typically occurs a decade later has cerebral involvement so dementia is more common and patient seldom survives a year (originally thought to be sporadic, but now known to be familial as well). •FFI pathology is characterized by severe selective atrophy of the thalamus. Alpers syndrome is the name given to prion diseases in infants. 3/26/2006
Page 1 and 2: 3/26/2006 3/26/2006 PRIONS Definiti
Page 3 and 4: Humans diseases • The modified fo
Page 5 and 6: 3/26/2006 Replication In normal cel
Page 7 and 8: Pathologies induced by prions The p
Page 9 and 10: Pathologies induced by prions These
Page 11: 3/26/2006 Prion Diseases Prion dise
Page 15 and 16: Humans might be infected by prions
Page 17 and 18: 2. PrPsc is associated with scrapie
Page 19 and 20: 4. Mutated gene can cause susceptib
Page 21 and 22: 3/26/2006 Evidence against the prio
Page 23 and 24: Treatment •PrPc overexpression fa

Handout 9 - Prions

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