Lecture 4: ECM

Question 1

Marfan Syndrome is a defect in what molecule?

Answer 1

Fibrillin I

Question 2

Fibril-Forming is what class of collagen?

Distribution?

Answer 2

Type I

Most connective tissue

Question 3

What are proteoglycans?

Answer 3

Repeating sugar chains with some protein mixed in

Question 4

Where is laminin found?

Role?

Answer 4

Basal lamina

Several domains with roles, forms networks

Think: “Networks”

Question 5

Junctional Epidermolysis Bullosa (JEB) contains a defect where?

Answer 5

Lamina V – Basement membrane

Question 6

What are the three binding sites on fibronectin?

Answer 6

1. Proteoglycan
2. Integrin
3. Collagen

Question 7

What collagen is affected in Osteogenesis Imperfecta?

Symptoms?

Answer 7

Collagen I

Mr. Glass

Question 8

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

Symptoms?

Answer 8

Collagen VII

Skin can slough off, blisters

Question 9

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

Answer 9

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

Question 10

What connects the two “trains” of fibronectin?

Answer 10

Dual disulfide bonds

Question 11

Which collagen has two end caps?

What are they?

Answer 11

Collagen IV

N-terminal tetramer
C-terminal dimer

Question 12

Anchoring Fibrils are what class of collagen?

Distribution?

Answer 12

Type VII

Attachments of basal laminae to underlying connective tissue

Question 13

What molecule gives elasticity to tissues?

What is an example of this?

Answer 13

Fibrillin

Aorta flexibility to accodate changing volume

Question 14

Nervous Tissue?

Answer 14

Conductive tissue that distributes signals to control bodily functions

Question 15

What are main STRUCTURAL classes in the ECM?

Answer 15

Collagen, Proteoglycans, Fibrillin

Question 16

What is an important role of fibronectin?

Answer 16

Blood clotting

Hint: Think “adhesive” proteins

Question 17

What is the role of fibroblasts

Answer 17

Secrete proteins

Question 18

Connective tissue?

Answer 18

Cells and extracellular matric that support and connect other basic tissues

Include bone, cartilage, and blood

Question 19

What do proteoglycans bind to?

Once bound, what comes next?

What can errors in this lead to?

Answer 19

Fibroblast Growth Factor (FGF)

Presents structure to receptor

Disorders such as arthritis

Question 20

What gives flexibility to Collagen IV?

Answer 20

Breaks in triple helix

Question 21

What is upregulated during proliferative stage of wound repair?

Answer 21

Collagen III, fibronectin, MMP, proteoglycans

Question 22

Is epithelia vascular?

Answer 22

No, capillaries run beneath basement membrane

Question 23

What collagen is affected in Alport Syndrome?

Symptoms?

Answer 23

Collagen IV

Vision, hearing, kidneys impaired

Question 24

How are collagen fibrils arranged?

Answer 24

Perpendicular

Question 25

Which collagen has one end cap? Which is it?

Answer 25

Collagen VII (fibril like) NC-1 stays on

Question 26

What is downregulated during remodeling stage of wound repair?

Answer 26

Collagen III, fibronectin Hint: Opposite of proliferative phase

Question 27

What structure is affected by DEB?

Answer 27

Anchoring fibrils, Collagen VII

Question 28

What is unique about Disaccharide Glycosaminoglycans (GAGs)? What does this result in?

Answer 28

Negatively charged from Sulfur Results in attractive of sodium (+), water follows--gives strength and lubrication

Question 29

What are two layers of basement membrane? Role?

Answer 29

Basal lamina and reticular lamina Holds cells to connective tissue, guide for cell migration during development

Question 30

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

Answer 30

MMP-2 (metalloproteinase)

Question 31

Type VII Collagen forms what?

Answer 31

Anchoring fibrils

Question 32

What is role of heparanase?

Answer 32

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

Question 33

Epithelium? Responsible for?

Answer 33

Sheets of cells covering surface or lining of cavity Responsible for major functions of most organs

Question 34

What is upregulated during inflammatory stage of wound repair?

Answer 34

Fibronectin, MMP

Question 35

What are main ADHESIVE classes in the ECM?

Answer 35

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

Question 36

What defect in regulation can cause improper wound healing?

Answer 36

Excessive MMP activation

Question 37

Why are epithelia said to be polar?

Answer 37

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

Question 38

Network-Forming is what class of collagen? Distribution?

Answer 38

Type IV Basal laminae

Question 39

What does fibrillin I surpress? What happens if there is a problem with this chain?

Answer 39

TGF, which inhibits growth factors. Structures will continue to grow, such as Marfan Syndrome

Question 40

What is upregulated during remodeling stage of wound repair?

Answer 40

Collagen I, MMP

Question 41

Type IV Collagen forms what?

Answer 41

Network forming

Question 42

What are two forms of fibronectin?

Answer 42

Matrix -- Tied down | Plasma -- Free floating

Question 43

What is the rough structure of collagen?

Answer 43

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

Question 44

Muscle?

Answer 44

Contractile tissue