Huntington Pathology - Krafts

Question 1

Pathogenesis of HD/

Answer 1

Loss of neurons in basal ganglia

• spiny striatal neurons would normally dampen movement
• result is choreoathetosis

Loss of cortex neurons
-cognitive cahnges

Question 2

Gross changes of HD in the brain?

Answer 2

• Small brain
• Atrophic striatum and cortex
• dilated lateral and third ventricles

Question 3

Microscopic features of HD

Answer 3

Severe loss of striatal neurons
Extensive fibrillary gliosis
Degree of degeneration directly related to severity of clinical symptoms

Huntingtin protein aggregates (NOT TYPO)
(probably is toxic to neurons)

Question 4

Why is ubuquitin an effective staining method for looking at Huntington’s pathology?

Answer 4

NO great stain for Huntingtin
Have a great stain for ubiquitin (which tags abnormal protein for removal)
These aggregates never get removed so you can see where they all are with ubuquitin