PPT Slide

Polyglutamine Neurodegenerative Disorders

• At least eight are currently known (HD, DRPLA, SCA-1, 2, 3, 7, 17, and SBMA)

• DRPLA is most like HD

• Affected brain regions include cortex, basal ganglia, brainstem, cerebellum and spinal cord

• The genes share no homology except for the poly-Q repeats

• The androgen receptor is a DNA-binding transcription factor

Ross, Neuron 1995

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