Loss of GABA Neurons

Huntington's disease is an autosomal-dominant disease in which GABAergic projection neurons in the striatum are destroyed. Early symptoms include mood changes progressing to uncontrolled movements (chorea) and eventually to loss of movement and death. Disease progression can be correlated with the loss of three separate populations of GABAergic striatal neurons, although these are not the sole casualties. Cortical neuronal loss occurs later in the disease as well.

The gene for Huntington's disease codes for the huntingtin protein, which contains a long polygluta-mine stretch. Normally, the number of glutamines in this protein is less than 40. Huntingtin mutations that include more than 40 glutamines are pathogenic. The number of glutamines is inversely related to the age of onset, and people with longer repeat lengths develop Huntington's disease at earlier ages. The function of the normal huntingtin protein is unknown, as is the reason for the susceptibility of GABAergic projection neurons.

The Huntington's disease gene is expressed throughout life, but disease symptoms normally begin in the fourth decade. This delay in symptom onset may reflect the ability of the brain to compensate for dysfunction for a period of time. Currently, there is no cure for Huntington's disease, and treatments are limited to palliative care.

Adult Dyslexia

Adult Dyslexia

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