GE CLST 73B Lecture 12: Lecture 12: Basal Ganglia (LEVINE)

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Functions: movement preparation, movement selection, plan movement execution, sequencing movement, performing remembered movements. Copper builds up in the basal ganglia: globes palidus/striatum. A genetic disorder, autosomal recessive disorder (two chromosomes that have the mutation, the genes that are not on the sex chromosomes) Both parents have to have the genes. This discovery of the disease led to discrimination between: Pyramidal system and extrapyramidal/prepyramidal system: huntington"s disease. Monogenetic disorder which leads to deterioration/neuronal degeneration of parts of striatum and cortex. Progressive course with fatal outcome (gets worse) Get very thin because their metabolism is very high. In venezuela were able to locate the gene that was abnormal. Expanded polyglutamine repeat (cag) causes translation of an elongated huntington protein with a toxic gain of function scientists want to stop gene from producing gene. Age-related neurons generative disease, characterized by the loss of dopamine rigid neurons in the substantial nigra. Pre-cursor dopamine (l-dopa, first treatment of parkinson"s) will cross blood-brain barrier.

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