Neurodegenerative diseases

Question 1

huntingtons basics

Answer 1

neurodegenerative disease in which neurones in the brain become damaged and die
- loss of mental and physical abilities

Question 2

which gene is abnormal in huntingtons

Answer 2

Htt (huntingtons gene)

Question 3

HTT gene in normal person

Answer 3

has 10-35 CAG repeats

Question 4

CAG codes for

Answer 4

glutamine

Question 5

HTT gene in those with the disease

Answer 5

people with mutated Htt have more than 36 CAG repeats

Question 6

more than 36 CAG repeats

Answer 6

abnormal stretch of polyglutamine alter the structure of the HTT protein and causes misfolding

Question 7

misfiling of Htt is

Answer 7

toxic to nerve cells and causes neuronal death in the basal ganglia

Question 8

how many chromosomes must be abnormal in huntingtons

Answer 8

1- autosomal dominant

Question 9

Alzheimer disease is caused by

Answer 9

the accumulation of B-amyloid plaques

Question 10

In healthy brain B-amyloid plaques are

Answer 10

broken down

Question 11

in alzheimers B-amyloid plaques

Answer 11

accumulate and form hard insoluble plaques

- these plaques then form between neurones in the brain

Question 12

Prions diseases are caused by

Answer 12

pyrion proteins

Question 13

pyrion proteins

Answer 13

cause proteins to fold abnormally

- cause spongiform encephalopathies

Question 14

examples of pyrion diseases

Answer 14

Creutzfel-Jakob - most common in humans

BSE- Mad cow

Question 15

Parkinson’s caused by

Answer 15

the misfolding of alpha-synuclein

Question 16

misfiling of alpha-synuclein in parkinsons causes

Answer 16

lewy bodies to form= causes death of dopaminergic neurones

Question 17

anticipation caused by

Answer 17

caused by trinucleotide repeat expansion

Question 18

number of trinucleotide repeats

Answer 18

increases as the gene is passed form parent to child meaning disorders become more severe with each generation