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Pathology > Collagen > Flashcards

Collagen Flashcards

1
Q

What is the most abundant protein in the body?

A

Collagen

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2
Q

What is the structure of collagen?

A 
Three alpha chains
wind around each other to give a triple helix
come together to form microfibrils
come together to form fibrils
come together to form fibres
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3
Q

Which types of collagen are the “fibrillar” ones?

A

Types 1-3

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4
Q

What is the role of the fibrillar collagens?

A

Give strength to tissues

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5
Q

Which types of collagen are the “amorphous” ones?

A

Types 4-6

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6
Q

What is the most common type of collagen in the body?

A

Type 1

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7
Q

Where is type 1 collagen found in the body?

A

Hard tissues - bone

Soft tissues - tendons, ligaments, skin, blood vessels, hollow organs, sclera

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8
Q

Where is type 4 collagen found?

A

Basement membrane

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9
Q

What secretes type 4 collagen in the basement membrane?

A

Epithelial cells

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10
Q

How long does collagen synthesis take?

A

1-2 hours

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11
Q

What enzyme is responsible for collagen remodelling?

A

Collagenase

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12
Q

Give an example of an acquired disease due to defective collagen synthesis

A

Scurvy

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13
Q

Give examples of three inherited diseases due to defective collagen synthesis

A

Ehler’s Danlos syndrome
Osteogenesis imperfecta
Alport syndrome

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14
Q

What is the cause of scurvy?

A

Vitamin C deficiency

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15
Q

How does vitamin C deficiency affect collagen synthesis?

A

Reduced hydroxylation of procollagen alpha chains

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16
Q

How does vitamin C deficiency affect the strength of collagen?

A

Reduced cross-linking between chains, microfibrils, fibrils

weaker collagen

17
Q

Collagen in ……………………. is particularly affected

A

Blood vessels

18
Q

What are the symptoms of scurvy?

A

Porlonged bleeding
Tooth loss
Old scars becoming fresh wounds

19
Q

Why does prolonged bleeding occur with scurvy?

A

Unale to heal wounds

20
Q

Why does tooth loss occur with scurvy?

A

Collagen in perodontal ligament has short half-life

replaced by defective collagen

21
Q

Why do old scars open up as fresh wounds with scurvy?

A

High collagen turnover in scars for 2 years after their formation
replaced by defective collagen

22
Q

What is the cause of Ehler’s Danlos syndrome?

A

Defective conversion of procollagen to tropocollagen

23
Q

How are collagen fibres different in Ehler’s Danlos syndrome?

A

Lack tensile strength, are weak

24
Q

What are the features of skin in Ehler’s Danlos syndrome?

A

Hyperextensible, recoils
Thin
Fragile
Susceptible to injury

25
Q

Why is the skin hyperextensible?

A

Elastin fibres stretch a lot, not held in check by tethering effect of collagen
then recoil

26
Q

What are the features of joints in Ehler’s Danlos syndrome?

A

Hypermobile

Predisposed to dislocation

27
Q

What are the complications of Ehler’s Danlos syndrome?

A

Rupture of colon
large arteries
cornea

Retinal detachment

28
Q

What is the cause of osteogenesis imperfecta?

A

Lack fo type 1 collagen

29
Q

What are the features of osteogenesis imperfecta?

A 
Fragile bones
Deformation of bones - progressive, severe
Blue sclera
Dental abnormalities
Hearing impairment
30
Q

Why are bones fragile and deformed in osteogenesis imperfecta?

A

Lack of bone tissue
cannot withstand mechanical stress
break easily, deformed easily

31
Q

Why is the sclera blue with osteogenesis imperfecta?

A

Lack of collagen within

so it’s translucent

32
Q

Which chromosome is faulty in Alport syndrome?

A

X chromosome

33
Q

Which gender is more commonly affected by Alport syndrome?

A

Males

34
Q

What is the cause of Alport syndrome?

A

Defective type 4 collagen

35
Q

How do people with Alport syndrome present?

A

Haematuria when children

Renal failure as adults

36
Q

What are the features of Alport syndrome?

A

Dysfunction of glomerular basement membrane
lens of eye
cochlea of ear

37
Q

What can the dysfunctional glomerular basement membrane lead to?

A

Kidney failure

38
Q

What can the dysfunctional lens of the eye lead to?

A

Eye disorders

39
Q

What can the dysfunctional cochlea of the ear lead to?

A

Neural deafness

Pathology (4 decks)

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