Types of collagen, synthesis, and diseases Flashcards Preview

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Flashcards in Types of collagen, synthesis, and diseases Deck (43):
1

Granulation tissue

III

2

OI results from impairement of:

Alpha triple helix formation during collagen synthesis

3

Skin

 

Mainly I, some III

3

What cofactor is required for hydroxylation of proline and lysine during collagen synthesis?

Vitamin C

3

In what form is collagen exocytosed into ECM during synthesis?

Procollagen (already in triple helix)

3

Most common type of Ehlers-Danlos?

Hypermobile type (joint instability)

4

Where is the alpha chain triple helix of precollagen formed?

RER of fibroblast

5

Cornea

I

6

Consensus sequence of collage

Gly-X-Y

6

Best reflection of collagen synthesis?

Glycine uptake by fibroblasts

7

Where is type IV collagen found?

Basement membrane, basal lamina, lens

7

What causes Menke disease?

Lack of copper transport and absorption --> no copper for lysyl oxidase cross-linking of lysine-hydroxylysine

8

Fascia

I

9

Where is type I collagen found?

Bone, skin, tendon, fascia, dentin, cornea, late wound repair

10

Kinky hair + growth retardation + hypotonia

Menke disease

11

Lens

IV

11

Which preprocollagen residues are hydroxylated in the RER of fibroblasts?

Proline and lysine

12

Uterus

III

13

Where is type III collagen found?

Blood vessels, granulation tissue, fetal tissue, uterus, skin

13

Hyperextensible skin + easy bruising/tendency to bleed + hypermobile joints

Ehlers-Danos

14

Basement membrane

IV

15

Multiple fractures + hearing loss + dental imperfections

OI

17

Tendon

I

19

Where is type II collagen found?

Cartilage (including hyaline), nucleus pulposus, vitreous body

19

Basal lamina

IV

21

Nucleus pulposus

II

22

Is type I collagen defective in OI?

No, just reduced production of otherwise normal type I collagen

23

Which residue of preprocollagen gets glycosylated in the RER of fibroblasts?

Hydroxylysine

24

Scurvy results in failure of which step of collagen synthesis?

Hydroxylation of proline and lysine in RER of fibroblast

26

Which type of collagen is underproduced in osteogensis imperfecta?

I

27

Name 5 diseases associated with collagen.

1. OI (no type I)

2. Ehlers-Danlos (no cross-linking)

3. Alport (defective IV)

4. Goodpasture's (autoantibodies against IV)

5. Menkes disease (no copper for lysyl oxidase)

29

Bone

I

30

Cartilage

II

32

Late wound repair

I

33

Risk of joint dislocation, aortic or berry aneurysms, organ rupture

Ehlers-Danlos

35

Blood vessels

III

36

Most common type of collagen

I

37

Which steps in collagen synthesis occur outside the fibroblast?

Cleave of terminal disulfide-bonds of procollagen to form tropocollagen

Cross-linking of lysine-hydroxylysine

38

Dentin

I

40

Which enzyme cross-links collagen? What is its co-factor?

Lysyl oxidase

Copper

41

Mode of inheritance: OI

AD

42

Fetal tissue

III

43

Vitreous body

II