PSB 3002 Lecture Notes - Lecture 25: Subthalamic Nucleus, Caudate Nucleus, Putamen
Document Summary
Cause: degeneration of dopamine neurons in susbtantia nigra. Lewy body: abnormal circular structures in cytoplasm of surviving dopamine neurons. Synuclein: presynaptic protein can form abnormal accumulations in dopamine neurons if mutated. One of the proteins is part of the ubiquitin-proteasome system) Toxic gain of functio(cid:374) ( - synuclein: ge(cid:374)eti(cid:272) disorder (cid:272)aused (cid:271)y do(cid:373)i(cid:374)a(cid:374)t mutation that involves faulty gene that produces protein with toxic effects. ---proteasome: orga(cid:374)elle respo(cid:374)si(cid:271)le for destroyi(cid:374)g defe(cid:272)tive or degraded protei(cid:374)s within the cell. ---ubiquitin: protei(cid:374) that attaches itself to faulty or misfolded proteins and thus targets them for destruction by proteasomes. Loss of function (parkin: ge(cid:374)eti(cid:272) disorder (cid:272)aused (cid:271)y re(cid:272)essive gene that fails to produce protein that is necessary for good health. Parkin: protein that plays role in ferrying defective or misfolded proteins to proteasomes, mutated parkin is cause of familial parkinson"s disease. --caused by degeneration of the caudate nucleus and putamen. --characterized by progressively more severe uncontrollable jerking movements, writhing movements, dementia, and finally death.
