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pH - Foundations 08-10-17 > ECM > Flashcards

Flashcards in ECM Deck (61):
1

GAGs

Glycosaminoglycans

2

Hyaluronan

GAG w/ no SO3

3

Keratan Sulfate

GAG w/ no acidic sugar

4

Core Protein w/ Hyaluronan

Aggrecan

5

GAGs Plus Core Protein

Proteoglycans

6

Why Are Proteoglycans Smaller Than GAGs?

They are bound to membrane

7

Name 3 Proteoglycans

1. Syndecan (spanning)
2. Glypican (GPI anchor)
3. Perlecan (secreted)

8

What Are the Most Abundant GAGs in the body? What do they make up?

1. Chondroitin 4 - Sulfate
2. Chondroitin 6 - Sulfate

Cartilage (bind collagen), tendons, ligaments, aorta

9

What GAG is in the skin, blood vessels, and heart valves?

Dermatan Sulfate

10

What is the most diverse GAG?

Keratin Sulfate (KS)

11

KS 1

Corneas

12

KS 2

Loose connective tissue with chondroitin sulfate

13

KS 3

Brain

14

Is Hyaluronate covalently bound to proteins? Where is it found? Involved in?

No
Bacteria, synovial fluid (lubricant) and shock absorber
Metastasis

15

Heparin (GAG)

Intracellular in mast cells (WBC) lining arteries (liver, lungs, skin) and is an anticoagulant

16

Heparin Sulfate (GAG)

Has acetylation (less sulfates). Extracellular GAG (basement membrane)

17

What does Heparin bond to?

Antithrombin 3

18

What are the 2 mucopolysaccharidose lysozyme storage diseases?

1. Hurler's Syndrome (developmental delays, gargoylism, airway obstruction, clouded cornea, hearing loss)
2. Hunter's Syndrome (mild Hurler + aggression)

Can't break down GAG's and can be delayed

19

What accumulates in Hurler's/Hunters?

Heparan Sulfate and Dermatan Sulfate

20

What enzyme is affected in Hunter's? What is the issue?

Iduronate Sulfatase. Cannot take off Sulfur (enzyme replacement theory)

21

What enzyme is affected in Hurler's? What's the issue?

Alpha-L-iduronidase. Cannot take off sugar

22

Sly Syndrome

No sugar off

23

How can Hurler's be treated?

Bone marrow or cord blood transplantation

24

What do you make collagen with?

a) 2 alpha 1's
b) 1 alpha 2

25

2 Patters in Collagen Helix

1. Gly-Pro-X
2. Gly-X-Hydroxyproline

26

Scurvy is what type of collagen impairment?

Fibril

27

Symptoms of scurvy?

Lethargy & malaise, bruises due to capillary fragility, gum disease, poor wound healing

28

Is Ehler's lethal? Most common a.a. mutations?

No; type 3 is most clinically significant (vascular) and type 5 is classical (joint and skin) also class 1

29

What type of collagen is affected in osteogenesis imperfecta and is it autosomal what?

Collagen type 1; autosomal dominant

30

What are 4 cell adhesion molecule families?

1. Selectins
2. Immunoglobulin Superfamily
3. Integrins
4. Cadherins

31

What ligand does selectins recognize? Is it a stable cell junction?

Carbs; no

32

What ligand does IG superfamily recognize? Stable cell junction?

IgSF members, integrins; not usually for either

33

What ligand does integrins recognzie? Stable?

EC matrix - focal adhesions
IG superfamily, Cadherins - hemidesmosomes

34

What ligand does cadherins recognize? Stable?

Other cadherins - adherens junctions, desmosomes

35

Which family does not require Ca+2, Mn+2, or Mg+2?

Ig

36

What motiff do integrins recognzie?

RGD (arginine, glycine, aspartic acid) any compound that has thing, integrins will bind

37

How many units in integrins? This relates to what?

24; metastasis

38

Where do integrins function?

Focal adhesions, hemidesmosomes

39

What is inside focal adhesions? Hemidesmosomes?

Actin filaments (talin, ECM); intermediate filaments basal lamina)

40

What does talin do? What else is involved?

Binds to integrin to activate it; kindlin

41

MMP

Matrixmetalloprotease; eats away at ECM to help cancer metastasize

42

What binds and activates MMP-2?

Alpha 5 beta 3 integrin

43

What 3 things do Adherins use?

1. Actin filaments inside; across cell using 1 protein each side
2. Beta Catenin
3. Alpha Catenin (the catenins help bind actin filament)

44

Desmosome uses what?

Intermediate filaments

45

2 places for tight junctions. Is it strong?

1. Epithelium
2. BBB

No; just separate 2 things in the body

46

What 3 things in tight interacts with actin? What do they bind?

1. JAM
2. Occludin
3. Claudin

PDZ

JOC (K)

47

Gap Junctions

Extension of the cell

48

Gap Junction Disease. Symtpoms?

Charcot-Marie-Tooth (CMT)

Degeneration of nerves, slow loss of muscle control, cataracts, deafness

49

What is fibronectin?

Matrix adhesions; crosslinks with GAGs and combines with integrins

50

What are the 2 types of fibronectins?

1. Insoluble (forming ECM as fibrils)
2. Soluble (blood - clotting)

51

What is Fibrosis?

Increased deposition of EC matrix proteins into tissues (scar, organ failure)

52

Fibrosis (45% of deaths in 2008) Blood Clotting

Fibronectin and fibrin cross link until collagen deposited (this is good)

53

Fibrosis =

Bad. Macrophages increase TGB beta, fibroblasts increase in matrix output - scarring

54

MMP's digest what? Involved in what?

Collagens, laminins; movement of cells, development/growth, tissue repair

55

When do MMP's increase?

Cancer

56

2 things that change in all cancers

Integrins and MMPs

57

Elastin mainly what?

Small, non-polar (rich in pro and gly)

58

Why not super rigid like collagen?

Crosslinks not a lot and can pull the crosslinks apart

59

What does smoking mess up for inhibition of elastase?

Methione in antitrypsin to bind elstase

60

What is fibrillin?

Glycoprotein scaffold for elastin

61

Marfan

Impaired structural activity