elm 3.2

Question 1

huntington’s disease is the destruction of______ ___

Answer 1

basal ganglia

Question 2

huntington’s disease is a _____ autosomal dominant disorder

Answer 2

inherited
(most common degenerative disease)

Question 3

what are some major symptoms of huntingtons?

Answer 3

cognitive problems
severe motor dysfunction
psychiatric problems

Question 4

huntington’s is a ______ disorder

Answer 4

trinucleotide repeat

Question 5

the outcome of huntingtons depends on the number of ____ repeats in huntington protein - the more repeats, the more severe the disease and earlier the onset

Answer 5

CAG (Q) - glutamine

Question 6

what is the normal number of CAG repeats to have?

Answer 6

<27
27-35 elevated

Question 7

mutant huntington is toxic to cells T or F

Answer 7

T

Question 8

mutation in huntington affects the whole brain, but ____ cells are more sensitive, and the indirect pathway _____ are lost first

Answer 8

basal ganglia
MSN (medium spiny neurons)

Question 9

the indirect pathway ____ mvmt

Answer 9

inhibits

Question 10

dopamine ____ mvmt

Answer 10

promotes

Question 11

in parkinson’s, ____ are pathogenic

Answer 11

lewis bodies

Question 12

tetrabenazine as a treatment for parkinson’s does what? (2)

Answer 12

inhibits vesicular monoamine uptake
decreases dopamine levels

Question 13

chloropromazine as a treatment for parkinson’s does what? (1)

Answer 13

dopamine antagonist
used as antipsychotic

Question 14

haloperidol, olanzapine, risperidone and quetiapine as a treatment for parkinson’s does what? (1)

Answer 14

dopamine antagonists

Question 15

mutant huntington is broken down differently to the normal proteins, and this results in what?

Answer 15

fragments containing extra glutamine repeats - thus have the propensity to misfold

Question 16

misfolded mHTT molecules can then what

Answer 16

aggregate to form inclusion bodies

Question 17

the presence of mHTT causes ____ dysfunction and triggers ____

Answer 17

mitochondrial
neuroinflammation

Question 18

in cholinergic neuron distribution, what are the two main cell groups?

Answer 18

pedunculopontine (PPT) and magnocellular forebrain

Question 19

in AD, a loss of cholinergic neurons from the _____ is marked and early

Answer 19

basal forebrain

Question 20

cholinesterase inhibitors _____ ACh availability

Answer 20

increase

Question 21

tau protein tangles can become _____

Answer 21

neurofibrillary tangles

Question 22

what kind of formation of amyloid plaques can there be?

Answer 22

non-amyloidogenic

amyloidogenic

Question 23

in non-amyloidogenic pathway, the amyloid precursor protein is first cleaved by ____

Answer 23

alpha secretase

Question 24

In the amyloidogenic pathway, APP is first cleaved by an enzyme called

Answer 24

beta-secretase

Question 25

of amyloidogenic and non-amyloidogenic pathways, which is safe and why?

Answer 25

non-amyloidogenic is safe bc it does not lead to formation of amyloid plaques

amyloidogenic is pathogenic bc it leads to production of amyloid beta peptides