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Molecular Basis Of Disease > Lecture 1 > Flashcards

Lecture 1 Flashcards

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1
Q

What does misfolding process trigger?

A

Self-association

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2
Q

What does self-association of proteins create?

A

Long amyloid plaques

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3
Q

What are proteins described as?

A

Living on a knife edge

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4
Q

Is the energy difference between folded and unfolded state huge?

A

No

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5
Q

What is the stability associated with a protein around?

A

5-15kcal/mol

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6
Q

What is one single hydrogen bond around?

A

2-5kcal/mol

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7
Q

How many hydrogen bonds does a medium sized protein have?

A

400-500 hydrogen bonds

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8
Q

What are Misfolding diseases a losing battle between?

A

Deposition and clearance

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9
Q

How many identified proteins are there that have caused different protein misfolding diseases?

A

24

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10
Q

What can diseases of amyloidosis be?

A

Local

Systemic

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11
Q

What is meant by local?

A

Restricted to a single organ e.g. brain

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12
Q

What is meant by systemic?

A

Protein aggregates can form across the whole body

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13
Q

What is half of your body mass?

A

A single aggregated protein

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14
Q

What are prions?

A

A group of conditions that affect the nervous system

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15
Q

Definition of prion

A

Collection of proteins which normally exist in our body but have misfolded and form insoluble aggregates

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16
Q

What does aggregation of proteins cause?

A

Death of cells

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17
Q

What is a PrP protein?

A

Normal protein found in the brain and under certain conditions e.g. mutation this protein will actually misfolding into protein PrPSC

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18
Q

What does PrP protein consist predominantly of?

A

Alpha helices

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19
Q

What does PrPSC have a higher proportion of?

A

Beta pleated sheets

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20
Q

What occurs during misfolding process?

A

Instead of forming all the alpha helices we form the beta pleated sheets

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21
Q

What does beta pleated sheets consist of?

A

Linear polymers that stack on top of one another - greater potential of bonding to other beta pleated sheets via non covalent bonds therefore forming aggregates

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22
Q

What are spongiform Encephalopathies prion disease?

A

Disease are fatal

No cure for them

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23
Q

What does spongiform refer to?

A

Characteristic appearance of infected brains which become filled with holes until they resemble sponges when examined under microscope

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24
Q

From early symptoms, when does the patient die?

A

Within a year

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25
What are the neuropathology of Spongiform Encephalopathies prion disease?
Spongiform degradation of the brain The brain shrinks and become spongy Leaves holes in the white and grey matter of the brain
26
What are aggregation of proteins called?
Amyloid plaques
27
What is the property of protein plaque?
Resistant to proteolytic degradation
28
What can prion diseases be?
Inheritable Sporadic Infectious
29
What are prions a devoid of?
Nucleic acid
30
When does cellular PrP converted into PrPSC?
When a portion of alpha-helical and coil structure is refolded into B-sheets
31
What is an example of sporadic disease?
Creutzfeldt-Jakob disease (CJD)
32
What is CJD?
Rare, degenerative fatal brain disorder
33
What are early stages of disease people may have of CJD?
Failing memory Behavioural changes Lack of coordination Visual disturbances
34
What happens with patients that have CJD as illness progresses?
``` Mental deterioration Involuntary movements Blindness Weakness of extremities Coma ```
35
What is Gertsmann-Straussler-Scheinker?
A type of prion disease
36
What is familial CJD?
Inherited form of CJD | Result from several types of mutation on prion gene
37
What is fatal familial insomnia?
Very rare sleep disorder that runs in families | Affects the thalamus
38
What does the thalamus control?
Emotional expression and sleep
39
What is main symptom of FFI?
Insomnia | Speech problems and dementia
40
Where did TSE come from?
Isolated tribe | The Fore-tribes-people in New Guinea
41
What is Kuru?
‘The trembling’ Neurodegenerative disease that gave a tremor Rare and fatal nervous system disease
42
How did the fore people contract the disease?
Performing cannibalism on corpses during funeral rituals
43
What are the symptoms of disease of Kuru?
``` Muscle twitching Loss of coordination Difficulty walking Involuntary movements Behavioural and mood changes Dementia Difficulty eating ```
44
What caused kuru?
The link between ritualistic cannibalism and kuru was a long incubation period (5 and 30 years)
45
What is cannibalism?
The practice of humans eating the flesh or internal organs of other human beings
46
What is Iatrogenic CJD?
Form of CJD which belong to a group of rare, fatal brain disorder called prion disease Arises from contamination with tissue from an infected person - medical procedure
47
How has CJD been transmitted?
Treatment with human growth hormone | Peripheral transmission
48
Human growth hormone
Treat children with short stature | Prepared from human pituitary gland
49
What is the incubation for transmitting with HGH?
3 and 20 years
50
Cornea and other grafts
Incubation 1 to 14 years
51
Scrapie in sheep
Most widespread form of TSE Transmissible and inheritable component to spongiform Encephalopathies Infectious but certain breeds are more prone to diseases
52
What is scrapie agent extremely resistant to?
Inactivation by UV and ionizing radiation
53
Is scrapie transmitted to humans?
No
54
How many cattle’s died from BSE?
> 160,000
55
How long does it take for symptoms of BSE to appear?
5 years
56
How many were infected with BSE?
1 million
57
What other animals did mad cow disease spread to?
Mink Mule Deer Cats
58
What is mad cow disease?
Neurodegenerative disease that can mutate and spread to humans
59
What causes mad cow disease?
Feeding cattle the remains of infected cows
60
What are the human symptoms of mad cow disease ?
Depression Apathy or anxiety Difficulty walking and controlling their limbs
61
What are the animal symptoms of mad cow disease?
Increasingly aggressive Lose control of movement Milk production stops Anorexia and legarthy
62
Sunday times, May 1990
Leading food scientist calls for slaughter of 6 million cows The UK government does not agree with him He is accused of ‘scare-mongering’
63
Daily Mirror, March 96
MAD COW CAN KILL YOU
64
Times June 96
Scientist find direct evidence for BSE link A French group injected macaque monkeys with BSE the pathology that was produced in their brain very similar to that found with patients that have died recently with vCJD
65
Oct 1996
The European Union has banned imports of British Beef | McDonald’s has pulled British Big Macs from its menu
66
New variant CJD
Only observed in UK and France Teenagers and young adults ~170 deaths
67
What is new variant CJD linked to?
Infected beef and the mad cow epidemic
68
What does recent work of new variant CJD show?
Mother to baby transmission | Blood transfusion: blood donor restrictions
69
What is vCJD not clustered to?
Particular area of UK

Molecular Basis Of Disease (7 decks)

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