ELM 3.2

Question 1

What is the normal number of CAG repeats in huntingtin?

a.	 <27

b.	 27-35

c.	 36-39

d.	 40+

Answer 1

a

Question 2

Which of the following drugs is most frequently used for early stage Huntington’s Disease?

a.	 Benserazide

b.	 Bromocriptine

c.	 Tetrabenazine

d.	 Tolcapone

Answer 2

c

Question 3

The loss of which neurons produces the chorea in early stage Huntington’s Disease?

a.	 Globus pallidus externus neurons projecting to the globus pallidus internus

b.	 Striatal neurons projecting to the globus pallidus internus

c.	 Striatal neurons projecting to the globus pallidus externus

d.	 Subthalmic nucleus neurons projecting to the globus pallidus internus

Answer 3

c

Question 4

Huntingtin is expressed:

a.	 in all parts of the body

b.	 only in the basal ganglia

c.	 only in medium spiny neurons

d.	 only in medium spiny neurons expressing D2 receptors

Answer 4

a

Question 5

There are two different forms of Aβ involved in Alzheimer’s Disease:

Answer 5

Aβ40 and Aβ42, the latter of which is thought to be more toxic.

Question 6

the presence of mutations in alpha beta appears to alter the breakdown of ____ so that the formation of the more toxic alphabeta___ is favoured

Answer 6

APP
42

Question 7

The presenilin proteins form part of the protease (____) that cleaves APP to yield ___. As with the APP mutations, mutations in presenilins appear to favour the formation of_____.

Answer 7

γ-secretase
Aβ
Aβ42

Question 8

the ____ gene is particularly important in alzheimers

Answer 8

APOE

Question 9

what does APOE code for?

Answer 9

E - apolipoprotein E

Question 10

why are people with down syndrome more likely to develop alzheimers?

Answer 10

down syndrome is trisomy of chromosome 21 - results in broad spectrum of physical and learning difficulties

gene for amyloid precursor protein is located on chromosome 21 therefore people with down syndrome have an extra copy of the gene are are likely to produce higher amounts of alpha beta protein

Question 11

early stages of alzheimers includes a loss of neurons from where

Answer 11

the forebrain
loss of cholinergic neurons from basal forebrain

Question 12

cholinesterase inhibitors increase what

Answer 12

ACh availability

Question 13

Which protease complex is presenilin a key component of?

a.	 α

b.	 β

c.	 γ

d.	 δ

Answer 13

c

Question 14

Which protein aggregates to form neurofibrillary tanges?

a.	 amyloid-β

b.	 alpha-synuclein

c.	 presenilin

d.	 tau

Answer 14

d

Question 15

Mutations in which protein are thought to explain a lot of the genetic risk in late onset sporadic Alzheimer’s Disease?

a.	 APOE

b.	 APP

c.	 Presenilin

d.	 Tau

Answer 15

a

Question 16

Which is the form of amyloid beta that is most closely associated with Alzheimer’s Disease?

a.	 Aβ36

b.	 Aβ39

c.	 Aβ40

d.	 Aβ42

Answer 16

d

Question 17

Which of the following drugs is an allosteric potentiator of nicotinic acetylcholine receptors?

a.	 Donepezil

b.	 Galantamine

c.	 Rivastigmine

d.	 Tacrine

Answer 17

b

Question 18

CJD is a ___ disease

Answer 18

prion

Question 19

In a prion disease, the infectious agent is a _______ that folds abnormally. It can induce other normally folded copies of the prion protein to take on the aberrant folding pattern. Because the misfolded protein has more _____ structure it has a greater tendency to ________. The misfolded proteins therefore form aggregates in______, leading to _______.

Answer 19

protein (prion protein, PrP)
beta-sheet
stick together
neurons
cell death