Extracellular matrix Flashcards

1
Q

What are the functions of the extracellular matrix?

A
Cellular division
Motility
Differentiation
Adhesion
Structure
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2
Q

What secretes the extracellular matrix?

A

Cell secretes macromolecules for ECM

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3
Q

What are the main components of ECM?

A

Structural proteins (Collagen, Elastin)

Proteoglycans (protein-polysaccharides)

Adhesive Glycoproteins (Fibronectins, Laminins)

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4
Q

What is the purpose for collagens & elastins in the ECM

A

Provide strength & flexibility

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5
Q

What is the purpose for proteoglycans in the ECM?

A

Provide matrix for collagens and elastins to embed within

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6
Q

What is the purpose of Adhesive glycoproteins in ECM?

A

Attach cells to matrix

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7
Q

How much of total body protein is collagen?

A

25-30%

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8
Q

Polypeptides of collagen

A

Triple helix of glycine, hydroxylysine, hydroxyproline

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9
Q

How much weight can collagen hold?

A

1mm fiber = 20 lbs

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10
Q

How many different collagen genes are there?

A

25 different alpha chains

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11
Q

What provides collagen with it’s strength?

A

Cross-linked collagen bundles

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12
Q

What causes loss of joint function during aging?

A

Abnormal collagen cross-linking

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13
Q

What is the symptom of collagen synthesis deficiency?

A

Hypermobility of joints

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14
Q

What is elastin made of?

A

Proteins with Glycine & Proline with no hydroxylation

Elastin is crosslinked between lysines

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15
Q

What causes wrinkles and inflexible joints?

A

Loss of elastin with aging

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16
Q

What is Supravalvular aortic stenosis? (SVAS)

A

Mutations in elastin gene disrupt elastin synthesis and result in narrowing of aorta or pulmonary vessels

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17
Q

WHat are proteoglycans?

A

Glycoproteins with glycosaminoglycans

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18
Q

What are the glycosaminoglycan side chains?

A

Chondroitin sulfate
Keratan Sulfate
Heparin

19
Q

What is the purpose for glycosaminoglycans?

A

Receptors, filtration, shock absorption, water retenetion

20
Q

What is hyaluronate?

A

Proteoglycan that acts as backbone in collagen

21
Q

What is the function of hyaluronate?

A

Lubricates any place with friction

22
Q

What is Heparin/Heparan SUlfate?

A

Heparan Sulfate is found in every cell body, role in inflammation

Heparin is an anticoaglant

23
Q

WHat is fibronectin?

A

Most common adhesive glycoprotein

Can be soluble, insoluble or partially soluble depending on splicing

24
Q

What is the main function for fibronectin?

A

Maintains cell shape

Blood clotting (soluble form)

Guides immune cells to wound

25
Q

What role does fibronectin play in cancer?

A

Carinomas are unable to synthesize fibronectin and cells lose their shape and detach from ECM

Giving fibronectin revert cells to normal shape and cells are no longer malignant

26
Q

Where are laminins located?

A

Basal laminae under epithelial cells in muscle, fat and schwann cells

Separates ECM from epithelial cells

27
Q

What is the function of the basal lamina?

A

Structural support

Selective permeability barrier

28
Q

Why is the basal lamina important in cancer?

A

May allow the migration of cancer cells

29
Q

What does disorded extracellular matrix remodeling lead to?

A

Scar formation

30
Q

What tissues does the ECM provide a structural matrix for

A

All tissues

Bone & Cartilage specifically

31
Q

Why is remodeling constant in the ECM?

A

Allows cells to be responsive to needs of the animal

32
Q

What sites and cells is the ECm most active?

A

Cartilage (Chondrocytes)
Bone (Osteocytes)
Tendon (fibrocytes)
Crnea (Fibrocytes)

33
Q

What are the parent cells of ECM and how do they regulate ECM?

A

Osteoblasts
Chondrocyte
Fibrocytes

Transcriptional regulation

34
Q

What do ECM cells respond to?

A

Paracrine signaling (Growth factors)

Physical signaling from other cells/ECM

35
Q

How is ECM degraded?

A

Metallproteinase enzymes

Serine proteases

36
Q

What are the characteristics of metalloproteinase?

A

Requires zinc or calcium

Specific for 1 ECM compoenent

37
Q

Are degradation enzymes auto-upregulated?

A

Yes, in pathways with a high turnover of ECM

38
Q

What are the Tissue Inhibitors of metalloproteinase?

A

TIMP 1,2,3,4

Irreversibly bind to metalloproteinase

39
Q

What are the Serine Protease inhibitors?

A

Alpha 1-antrypsin

40
Q

What is Alpha 1-antrypsin deficiency?

A

A lack of Alpha 1-antrypsin serine protease inhibitor leads to excessive degradation of elastin by the neutrophil elastase enzyme

41
Q

What diseases are caused by alpha 1-antrypsin deficiency?

A

Emphysema, COPD, cirrhosis

42
Q

Treatment of alpha 1-antrypsin deficiency?

A

Intravenous infusions of alpha 1-antrypsin

43
Q

What is panniculitis?

A

Alpha 1-antrypsin deficiency in dogs can cause hardening of the skin in to painful, hard lumps and patches

Can occur in a range of severity and age