Lecture 4: ECM Flashcards

Extracellular Matrix

1
Q

Marfan Syndrome is a defect in what molecule?

A

Fibrillin I

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2
Q

Fibril-Forming is what class of collagen?

Distribution?

A

Type I

Most connective tissue

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3
Q

What are proteoglycans?

A

Repeating sugar chains with some protein mixed in

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4
Q

Where is laminin found?

Role?

A

Basal lamina

Several domains with roles, forms networks

Think: “Networks”

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5
Q

Junctional Epidermolysis Bullosa (JEB) contains a defect where?

A

Lamina V – Basement membrane

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6
Q

What are the three binding sites on fibronectin?

A
  1. Proteoglycan
  2. Integrin
  3. Collagen
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7
Q

What collagen is affected in Osteogenesis Imperfecta?

Symptoms?

A

Collagen I

Mr. Glass

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8
Q

What collagen is affected in Dystrophic Epidermolysis Bullosa (DEB)?

Symptoms?

A

Collagen VII

Skin can slough off, blisters

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9
Q

What are the three stages (with times) of wound repair?

A
  1. Inflammatory (hours)
  2. Proliferative (hours-days-weeks)
  3. Remodeling (weeks-months)
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10
Q

What connects the two “trains” of fibronectin?

A

Dual disulfide bonds

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11
Q

Which collagen has two end caps?

What are they?

A

Collagen IV

N-terminal tetramer
C-terminal dimer

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12
Q

Anchoring Fibrils are what class of collagen?

Distribution?

A

Type VII

Attachments of basal laminae to underlying connective tissue

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13
Q

What molecule gives elasticity to tissues?

What is an example of this?

A

Fibrillin

Aorta flexibility to accodate changing volume

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14
Q

Nervous Tissue?

A

Conductive tissue that distributes signals to control bodily functions

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15
Q

What are main STRUCTURAL classes in the ECM?

A

Collagen, Proteoglycans, Fibrillin

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16
Q

What is an important role of fibronectin?

A

Blood clotting

Hint: Think “adhesive” proteins

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17
Q

What is the role of fibroblasts

A

Secrete proteins

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18
Q

Connective tissue?

A

Cells and extracellular matric that support and connect other basic tissues

Include bone, cartilage, and blood

19
Q

What do proteoglycans bind to?

Once bound, what comes next?

What can errors in this lead to?

A

Fibroblast Growth Factor (FGF)

Presents structure to receptor

Disorders such as arthritis

20
Q

What gives flexibility to Collagen IV?

A

Breaks in triple helix

21
Q

What is upregulated during proliferative stage of wound repair?

A

Collagen III, fibronectin, MMP, proteoglycans

22
Q

Is epithelia vascular?

A

No, capillaries run beneath basement membrane

23
Q

What collagen is affected in Alport Syndrome?

Symptoms?

A

Collagen IV

Vision, hearing, kidneys impaired

24
Q

How are collagen fibrils arranged?

A

Perpendicular

25
Q

Which collagen has one end cap?

Which is it?

A

Collagen VII (fibril like)

NC-1 stays on

26
Q

What is downregulated during remodeling stage of wound repair?

A

Collagen III, fibronectin

Hint: Opposite of proliferative phase

27
Q

What structure is affected by DEB?

A

Anchoring fibrils, Collagen VII

28
Q

What is unique about Disaccharide Glycosaminoglycans (GAGs)?

What does this result in?

A

Negatively charged from Sulfur

Results in attractive of sodium (+), water follows–gives strength and lubrication

29
Q

What are two layers of basement membrane?

Role?

A

Basal lamina and reticular lamina

Holds cells to connective tissue, guide for cell migration during development

30
Q

What protein structure allows degradation of matrix for ECM-mediated cell migration?

A

MMP-2 (metalloproteinase)

31
Q

Type VII Collagen forms what?

A

Anchoring fibrils

32
Q

What is role of heparanase?

A

Angiogenesis

Degrades Heparan Sulfate (HS), allows growth factor to bind to blood vessel

33
Q

Epithelium?

Responsible for?

A

Sheets of cells covering surface or lining of cavity

Responsible for major functions of most organs

34
Q

What is upregulated during inflammatory stage of wound repair?

A

Fibronectin, MMP

35
Q

What are main ADHESIVE classes in the ECM?

A

Fibronectin, Laminin

Hint: Nectar sticky like adhesive, laminin as in stick laminate to your floors

36
Q

What defect in regulation can cause improper wound healing?

A

Excessive MMP activation

37
Q

Why are epithelia said to be polar?

A

Substances, such as O2, CO2, and waste transfer to and from the surface layer

38
Q

Network-Forming is what class of collagen?

Distribution?

A

Type IV

Basal laminae

39
Q

What does fibrillin I surpress?

What happens if there is a problem with this chain?

A

TGF, which inhibits growth factors.

Structures will continue to grow, such as Marfan Syndrome

40
Q

What is upregulated during remodeling stage of wound repair?

A

Collagen I, MMP

41
Q

Type IV Collagen forms what?

A

Network forming

42
Q

What are two forms of fibronectin?

A

Matrix – Tied down

Plasma – Free floating

43
Q

What is the rough structure of collagen?

A

x-y-z repeating monomers combined in a triple helix

44
Q

Muscle?

A

Contractile tissue