The Extracellular Matrix Flashcards

1
Q

Why is the ECM important?

A
  • Architecture of natural tissues and organs
  • Bioenginerring and synthestic organs
  • Supports cells and affects mechanical properties
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the ECM?

A

An extracellular assembly of glycoproteins, collagens, and polysaccharides

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the 2 main forms of ECM?

A
  • Connective tissue ECM
  • Basement membrane
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the major components of the connective tissue ECM?

A
  • collagen based fibrils
  • fibrillin and elastin microfibrils
  • Fibronectin fibrils
  • Extracellular proteoglycans
  • Hyaluronan
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the connective tissue ECM?

A
  • 3D meshwork that surrounds mesenchymal cells or neurons
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What do collagen fibres give the connective tissue ECM?

A

High tensile strength

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What provides elasticity of connective tissue ECM?

A

Fibrillin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the features of fibrillar collagens?

A
  • Globular N and C terminals
  • Helical central domain with GXY repeats
  • 3 polypeptide helices come together
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What makes up mature collagen?

A
  • Only the helix region
  • N and C globular domains are cleaved
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What kind of helix does the helical domain of GXY repeats form?

A

Left handed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What kind of helix do the 3 polypeptides of protocollage form?

A

Right handed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the features of fibrillin-1?

A
  • Secreted protein with mnay EGF-like domains
  • 7 TB domains
  • Multimerises by crosslinks into micro-fibrils
  • Many binding sites for other ECM proteins
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are the functions of fibrillin-1?

A
  • Adds stretch/elasticity
  • Binding sites for TGF-β growth factors and controls their localisation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

In a relaxed state what kind of form is elastin in?

A

A disordered state

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How is TGF-β bound to microfibrills?

A

As a large latent complex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What happens to elastin when it is elongated under mechanical force?

A

The simple molecular structure becomes highly crosslinked

17
Q

How does TGF-β interact with microfibrils?

A
  • Binds to microfibrills in latent complex
  • Held in inactive state
  • Under certain conditions TGF-β is released and can activate gene expression for cell survival
18
Q

Under what conditions is TFG-β freed?

A
  • Proteases produced
  • Action of mechanical force on microfibrils
19
Q

What causes Marfan’s syndrome?

A

Mutations in Fibrillin-1

20
Q

What does Marfan’s syndrome affect?

A
  • Microfibril organisation and strength
  • Causes very flexible joints
  • Progressive cardiovascular, skeletal, or occular problems
21
Q

How does mutation in Fibrillin-1 cause the symptoms of Marfan’s syndrome?

A
  • Results in aberrant local activation of TGF-β and excess TGF-β signalling
  • TGF-β signalling regulates expression of ECM genes and ECM composition
  • Due to microfibril disortion weakening TGF-β more likely to be released