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Molecular Basis Of Disease > Lecture 1 > Flashcards

Lecture 1 Flashcards

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
Q

What are the neuropathology of Spongiform Encephalopathies prion disease?

A

Spongiform degradation of the brain
The brain shrinks and become spongy
Leaves holes in the white and grey matter of the brain

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26
Q

What are aggregation of proteins called?

A

Amyloid plaques

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27
Q

What is the property of protein plaque?

A

Resistant to proteolytic degradation

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28
Q

What can prion diseases be?

A

Inheritable
Sporadic
Infectious

29
Q

What are prions a devoid of?

A

Nucleic acid

30
Q

When does cellular PrP converted into PrPSC?

A

When a portion of alpha-helical and coil structure is refolded into B-sheets

31
Q

What is an example of sporadic disease?

A

Creutzfeldt-Jakob disease (CJD)

32
Q

What is CJD?

A

Rare, degenerative fatal brain disorder

33
Q

What are early stages of disease people may have of CJD?

A

Failing memory
Behavioural changes
Lack of coordination
Visual disturbances

34
Q

What happens with patients that have CJD as illness progresses?

A 
Mental deterioration 
Involuntary movements 
Blindness 
Weakness of extremities 
Coma
35
Q

What is Gertsmann-Straussler-Scheinker?

A

A type of prion disease

36
Q

What is familial CJD?

A

Inherited form of CJD

Result from several types of mutation on prion gene

37
Q

What is fatal familial insomnia?

A

Very rare sleep disorder that runs in families

Affects the thalamus

38
Q

What does the thalamus control?

A

Emotional expression and sleep

39
Q

What is main symptom of FFI?

A

Insomnia

Speech problems and dementia

40
Q

Where did TSE come from?

A

Isolated tribe

The Fore-tribes-people in New Guinea

41
Q

What is Kuru?

A

‘The trembling’
Neurodegenerative disease that gave a tremor
Rare and fatal nervous system disease

42
Q

How did the fore people contract the disease?

A

Performing cannibalism on corpses during funeral rituals

43
Q

What are the symptoms of disease of Kuru?

A 
Muscle twitching 
Loss of coordination 
Difficulty walking 
Involuntary movements 
Behavioural and mood changes 
Dementia 
Difficulty eating
44
Q

What caused kuru?

A

The link between ritualistic cannibalism and kuru was a long incubation period (5 and 30 years)

45
Q

What is cannibalism?

A

The practice of humans eating the flesh or internal organs of other human beings

46
Q

What is Iatrogenic CJD?

A

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
Q

How has CJD been transmitted?

A

Treatment with human growth hormone

Peripheral transmission

48
Q

Human growth hormone

A

Treat children with short stature

Prepared from human pituitary gland

49
Q

What is the incubation for transmitting with HGH?

A

3 and 20 years

50
Q

Cornea and other grafts

A

Incubation 1 to 14 years

51
Q

Scrapie in sheep

A

Most widespread form of TSE
Transmissible and inheritable component to spongiform Encephalopathies
Infectious but certain breeds are more prone to diseases

52
Q

What is scrapie agent extremely resistant to?

A

Inactivation by UV and ionizing radiation

53
Q

Is scrapie transmitted to humans?

A

No

54
Q

How many cattle’s died from BSE?

A

> 160,000

55
Q

How long does it take for symptoms of BSE to appear?

A

5 years

56
Q

How many were infected with BSE?

A

1 million

57
Q

What other animals did mad cow disease spread to?

A

Mink
Mule
Deer
Cats

58
Q

What is mad cow disease?

A

Neurodegenerative disease that can mutate and spread to humans

59
Q

What causes mad cow disease?

A

Feeding cattle the remains of infected cows

60
Q

What are the human symptoms of mad cow disease ?

A

Depression
Apathy or anxiety
Difficulty walking and controlling their limbs

61
Q

What are the animal symptoms of mad cow disease?

A

Increasingly aggressive
Lose control of movement
Milk production stops
Anorexia and legarthy

62
Q

Sunday times, May 1990

A

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
Q

Daily Mirror, March 96

A

MAD COW CAN KILL YOU

64
Q

Times June 96

A

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
Q

Oct 1996

A

The European Union has banned imports of British Beef

McDonald’s has pulled British Big Macs from its menu

66
Q

New variant CJD

A

Only observed in UK and France
Teenagers and young adults
~170 deaths

67
Q

What is new variant CJD linked to?

A

Infected beef and the mad cow epidemic

68
Q

What does recent work of new variant CJD show?

A

Mother to baby transmission

Blood transfusion: blood donor restrictions

69
Q

What is vCJD not clustered to?

A

Particular area of UK

Molecular Basis Of Disease (7 decks)

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