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POM 1: Molecular Biology Of The Cell > 2.9: Extracellular Matrix > Flashcards

2.9: Extracellular Matrix Flashcards

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1
Q

What forms the ECM (2)

A

Fibrillar and non-fibrillar components

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2
Q

What is the extracellular matrix

A

Complex network of proteins and carbohydrates filling space between cells

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3
Q

Fibrillar means

A

To produce fibres

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4
Q

Two roles of the ECM

A

-Architectural (structural role)
-Instructional (behaviours of the cell)

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5
Q

3 things ECM is essential for

A
  • development
  • tissue function
  • organogenesis
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6
Q

3 key functions of ECM

A

-physical support
-determines mechanical and physiological properties of tissue
-influences growth, adhesion, differentiation status of cells and tissues

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7
Q

What mainly makes up the ECM

A

Connective tissue

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8
Q

3 Main components of the ECM

A

-collagens
-multi-adhesive glycoproteins
-proteoglycans

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9
Q

3 components making up basement membrane of ECM

A
  • Type IV
  • Laminins
  • Perlecan
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10
Q

Properties of connective tissue in bone

A

Hard and dense

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11
Q

Properties of connective tissue in cartilage

A

Resilient and shock absorbing

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12
Q

Properties of connective tissue in tendons and skin

A

Tough and flexible

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13
Q

Reason behind various properties of connective tissues

A
  • different types of collagen
  • different arrangement of oriented collagen
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14
Q

What cell produces collagen

A

Fibroblasts

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15
Q

What is connective tissue made up of

A

ECM and component cells

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16
Q

3 proteoglycans

A

Aggrecan
Versican
Decorin

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17
Q

2 multi-adhesive glycoproteins

A

Fibronectin
Fibrinogen

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18
Q

What type of protein is collagen

A

Fibrous

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19
Q

How many different types of collagen are there in humans

A

28

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20
Q

Structure of each collagen molecule

A

Three a chains forming a triple helix

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21
Q

Chains in Type I collagen structure

A

3 chains from two different genes

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22
Q

What is a homotrimer

A

Collagen has one chain type

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23
Q

What types of collagen are homotrimers

A

Type II
Type III

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24
Q

What type of collagen is a heterotrimer

A

Type 1

25
Q

What is commonly the x and y of the glycine-x-y repeat

A

X = proline
Y= hydroxyproline

26
Q

What amino acid occupied every third position on in the alpha helix chain of collagen

A

Glycine

27
Q

What property of aggrecan allows it to resist compressive forces as part of the cartilage?

A

High negative charge —>
High degrees of GAG sulfation with large numbers of negatively charged carboxyl groups attracts cations (Na+ ). large quantities of water retained by the highly negatively charged environment, resist compressive forces.

Under compressive load, water is given up by aggrecan, but regained once the load is reduced.

28
Q

Which of the following are potential features of Marfan’s syndrome? (Due to Mutations in the protein fibrillin-1)

A

Predisposition to aortic ruptures

29
Q

Why does the amino acid glycine feature so frequently in the primary sequence of a collagen chain?

A

Its small sidechain occupies the interior of the triple helix, allowing for tight packing

30
Q

Composition of type 1 collagen

A

[a1(I)]2[a2(I)]

31
Q

What provides tensile strength and stability in collagen?

A

Crosslinking

32
Q

When does cross linking in collagen take place?

A

Following collagen secretion

33
Q

What is an essential post translational modification which contributes to interchain H bond formation?

A

Hydroxylation of proline and lysine

34
Q

What enzymes are required for the hydroxylation of lysine and proline

A

Lysyl hydroxylase
Prolyl
(require Fe2+ and Vit C)

35
Q

What happens to collagen when you have a lack of Vit C (deficiency)

A

Collagen underhydroxylated

36
Q

When does collagen cross linking occur

A

After fibril formation

37
Q

2 fibril associated collagens

A

Type IX (9)
Type XII (12)

38
Q

Function of fibril associated collagens

A

Regulate organisation of collagen fibrils

39
Q

Type IV collagen characteristics (2)

A
  • network-forming collagen
  • in all basement membranes (structure varies from tissue to tissue)
40
Q

Symptoms of Ehlers-Danlos syndrome

A
  • stretch skin
  • loose joints
    Due to mutations affecting collagen prod. In connective tissue
41
Q

How is tensile strength in collagen established?

A

Fibres held in parallel bundles resisting tensile force in one direction

42
Q

What are collagen alpha chains synthesised from?

A

Pro-alpha chains (Longer precursors)

43
Q

What is cleaved from procollagen to make collagen?

A

N-terminal and C-terminal propeptides

44
Q

Where is type 4 collagen found

A

Basement membrane

45
Q

What type of network does type 4 collagen from?

A

Sheet-like network

46
Q

What happens to the N and C terminus in type 4 collagen?

A

Remains intact

47
Q

Alternate name for basement membrane

A

Basal lamina

48
Q

What is the basal lamina

A
  • thin, flexible, mat of extra-cellular matrix which epithelial cell sheets sit on top of
49
Q

3 structures basement membrane surrounds

A
  • muscle
  • peripheral nerve
  • fat cells
50
Q

How is the basement membrane involved in kidney ultrafiltration

A

Form the glomerular basement membrane

51
Q

What is diabetes nephropathy?

A

Disorder where accumulation of ECM leads to highly thickened basement membrane

52
Q

What is Alport Syndrome?

A

Mutations in Collagen IV
Result in abnormally laminated Glomerular basement membrane
Loss of kidney function and hearing loss

53
Q

What helps decrease extent of electric fibres stretching?

A

Fibres interwoven with collagen

54
Q

What are elastin fibres composed of?

A

Elastin protein core, surrounded by microfibrils (protein rich — fibrilin)

55
Q

Amino acid covalently cross linked in elastin

A

Lysine

56
Q

Where is fibrillin found

A

In microfibrils surrounding the elastin core

57
Q

Mutations in Fibrillin-1 are associated with ______ syndrome

A

Marfans syndrome

58
Q

Individuals with Marfans syndrome are more predisposed to….

A

Aortic ruptures

POM 1: Molecular Biology Of The Cell (2 decks)

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