BIOL 200 Lecture Notes - Lecture 35: Ern1, Secretion, Endoplasmic Reticulum
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Later onset than alzheimer"s; affected individuals show no symptoms for majority of life. Strange plaques or tangles begin to form in brain; may be associative or causative with relation to disease o disease can occur without plaques, plaques can occur without disease. There may be threshold of plaques needed before disease occurs, or disease must occur before plaque increases. In many neurodegenerative diseases, plaques are not observed o kuru. Prion protein (prp) proteins that may serve some physiological role. Exists in conformer, non-infectious state (prpc) and non-conformer, resistant state (prpsc) Act dominantly to disrupt correct folding of proteins. Prpsc can cause prpc proteins to misfold accumulation cellular dysfunction; causes infection in dominant manner. People in new guinea tribe consume remains of deceased loved ones, including neural remains. Kuru associated with number of neurological pathological: sudden outbursts of uncontrolled laughter, crossing of eyes, eventual palsies.