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ECM Flashcards

1
Q

What are the three basic ground elements of the ECM?

A

Ground substance
Fibers
Granulation tissues

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2
Q

What are the characteristics of the ground substance of the ECM?

A

amorphous, tranparent, colorless, and homogenous

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3
Q

What are the functional attributes of the ECM?

A 
Structure
Defense
Nutrition
Diffusion of gases, molecules, ions
Cell growth, survival
Cell migration
Lubrication
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4
Q

Where is lubrication used?

A

cartilage (joints)

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5
Q

What does the ground substance include?

A

GAGs (= mucopolysaccharides)

Proteoglycans

Water

Adhesive glyoproteins

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6
Q

What are the constituents of the fibers?

A

Collagen

Reticular (type III collagen)

Elastic

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7
Q

What are the two type of cell repair?

A

Granulation tissue (replacing dead cellswith ECM) (scar)

Replace functional tissue

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8
Q

How does the ECM drive the cell cycle?

A

Senses points of contact, such that more contacts will increase prb of growth

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9
Q

What is the probability of a cell suspended in agar of entering S phase?

A

8%

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10
Q

What determines if a tissue will replace damage with scar tissue or cells?

A

Whether or not the ECM is damaged–if it is not, then replace with cells.

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11
Q

What causes the attraction of water to GAGs?

A

Carboxyl groups

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12
Q

What are the six types of GAGs?

A 
Dermatan sulfate
Chondrotin sulfate
Heparan sulfate
Keratan sulfate
Hyaluronic acid
Heparin
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13
Q

What causes the increased hydration of tissue behind the eyes in grave’s disease?

A

Chrondroitin sulfate and hyaluronic acid deposition

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14
Q

What is the structure of proteoglycans?

A

Thistle brush

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15
Q

What is the backbone of proteoglycans? Branches?

A

Hyluronic acid

GAGs are branches

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16
Q

What is syndecan?

A

Transmembrane proteoglycan that connects FGF receptors via GAG branches (coreceptor for fibroblast growth factor)

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17
Q

What is perlecan?

A

Proteoglycan present in the basement membrane of tissues involved in proliferation, differentiation etc.

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18
Q

Inactivation of perlecan results in what?

A

Skeletal dysplasia

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19
Q

What are the 3 typical binding domains for multiadhesie glycoproteins?

A

cell adhesion molecules (CAMs, e.g. integrin)
Collagen fibers
Proteoglycans

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20
Q

Over hydration of the ground substance leads to what?

A

Edema

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21
Q

The ground substance fill space between what and what?

A

Cells and fibers

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22
Q

What are proteoglycans?

A

Linear chains of glycosaminoglycans bound to a protein core form proteoglycans

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23
Q

What percent of the molecular weight of a proteoglycan is due to carbs?

A

80-90%

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24
Q

What are glycosaminoglycans?

A

Long chains of unbranched poly saccharides consisting of a repeating disaccharide unit

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25
Q

What is the substance that forms the “core” of the proteoglycans?

A

Hyaluronic acid

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26
Q

What are the function of perlecans?

A 
Cell adhesion
Cell proliferation
Cell differentiation
Glomerular filtration
Development
Growth factor binding
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27
Q

What is the probability of a cell dividing when it is perched on a small adhesive patch?

A

30%

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28
Q

What is the probability of a cell dividing when it is perched on a LARGE adhesive patch?

A

90%

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29
Q

When GAGs form a bond with Hyluronic acid, what does it then become?

A

Aggregans

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30
Q

What is a major consequence of aggregan formation?

A

Increases hydration state

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31
Q

What are aggregans attached to?

A

Type I collagen

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32
Q

What are multiadhesive glycoproteins?

A

are glycoproteins that molecularly tether the cell to extracellular elements

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33
Q

What are the three primary binding sites for multiadhesive glycoproteins?

A

one for integrins (cell adhesion molecules), one for collagen fibers, and one for proteoglycans

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34
Q

What is the binding domain and location of fibronectin?

A

BInds to integrins, collagen, heparin, heparan sulfate, hyaluronic acid

Location = connective tissue, blood plasma, and embryonic tissue

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35
Q

What is the binding domain and location of Laminin?

A

Binds to integrins, heparane sulfate, collagen IV, and entactin

Found in the basal lamina

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36
Q

What is the binding domain and location of Tenascin?

A

Binds to syndecans and fibronein

Location = basal lamina

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37
Q

What is the binding domain and location of chondronectin?

A

Binds to collagen, II, chondroitin sulfates, hyaluronic acid, integrins of chondrocytes

Location = carilage

38
Q

What is the binding domain and location of osteonectin?

A

Binds to collagen, proteoglycans, and integrins of bone cells

Location = bones

39
Q

Blocking fibronectin blocks what important development process in mice?

A

mophogenesis

40
Q

What is the most abundant protein in the body?

A

Collagen (20-25% of all protein)

41
Q

What are the three major amino acids that make up collagen?

A

Glycine, hydroxyproline, hydroxylysine

42
Q

Where is type I collagen found?

A

Dermis, tendon, bone, dentin, cementum, fibrocartilage, organ capsules

43
Q

Where is type II collagen found?

A

Hyaline and elastic cartilage

44
Q

Where is type III collagen found?

A

Spleen, liver, lymph nodes, smooth muscle, skin, lung

45
Q

Where it type IV collagen found?

A

Basal lamina

46
Q

Where is collagen I synthesized?

A

Fibroblasts, odontoblasts, cementoblasts, osteoblats, chondroblasts

47
Q

Where is collagen II synthesized?

A

Condroblasts

48
Q

Where is collagen III synthesized?

A

Smootuh muscle cells, fibroblasts, reticular cells

49
Q

Where is collagen IV synthesized?

A

Endothelial cells, epithelial cells, schwann cells, smooth muscle cells

50
Q

What is the function of type I collagen?

A

Resists tension

51
Q

What is the function of type II collagen?

A

resistes pressure

52
Q

What is the function of type III collagen?

A

maintains structure in expansible organs

53
Q

What is the function of type IV collagen?

A

Supports delicate structures

Filtration

54
Q

what is the molecular organization of type I collagen?

A

Fibril-forming collagen leading to the formation of fibers

55
Q

what is the molecular organization of type II collagen?

A

Fibril-forming collagen

56
Q

what is the molecular organization of type III collagen?

A

Fibril-forming collagen leading to the formation of fibers

57
Q

what is the molecular organization of type IV collagen?

A

Network-forming collagen

58
Q

Where is type VII collagen found?

59
Q

What types of cells synthesize type VII collagen?

A

epidermal cells

60
Q

What is the function of type VII collagen?

A

anchors the lamina densa to the lamina reticularis

61
Q

What is the molecular organization of type VII collagen?

A

Anchoring collagen

62
Q

What amino acid is measured to calculate the [C] of collagen?

A

Hydroxyproline

63
Q

Is collagen acidophilic or basophilic?

A

Acidophilic

64
Q

What are the two means collagen can be degraded?

A

Proteolytic

Phagocytic

65
Q

Trichrome methods will color collagen what color?

66
Q

Reticular fibers are composed of what?

A

Type III collagen

67
Q

What do reticular fibers stain with?

A

Argyrophilic (silver) and PAS

68
Q

What types of cells synthesize retiular fibers?

A

fibrocytes, reticulocytes, smooth myocytes, and schwann cells

69
Q

True or false: reticular fibers branch?

70
Q

What is the function of elastic fibers?

A

Imparts tissue with elasticity

71
Q

Which stain would you use to highlight elastic fibers?

A

Verhoeff or resorcin-fushsin

72
Q

How are elastic fibers arranged?

A

b. Densely arranged in bundles (ligamentum flavum)

73
Q

What are the three components of elastic fibers?

A

Proelastin (contains a.a. desmosine and isodesmosine)

Microfibril-associated glycoproteins

Fibrillin (1 and 2)

74
Q

Which amino acids are unique to the ECM?

A

Desmosine and isodesmosine

75
Q

What is the defect and clinical relevance of Ehlers-Danlos type 4?

A

Mutation in the COL3A1 gene encoding type III collagen (reticular fibers)

Varicose veins, aortic rupture, intestinal rupture

76
Q

What is the defect and clinical relevance of Ehlers-Danlos type 6?

A

Defective hydroxylation of lysine thereby destabilizing the strength of the collagen

Hyperelasticity of the skin, rupture of the eyeball

77
Q

What is the defect and clinical relevance of Ehlers-Danlos type 7?

A

Mutations in the COL1A1 and COL1A2 genes encoding type I collagen

Joint dislocation and hypermobility of joints

78
Q

What is the defect and clinical relevance of osteogenesis imperfecta?

A

Mutations in COL1A1 lead to a reduction in the synthesis of type I collagen

Spontaneous fractures and cardiac insufficiency

79
Q

What is the defect and clinical relevance of strickler syndrome?

A

Mutation in COL2A1 gene encoding for type II collagen

Myopia, hypoplasia of mandible, arthritis

80
Q

What is the defect and clinical relevance of Marfan syndrome?

A

Mutation of the fibrillin 1 gene located on chromosome 15

Aortic aneurysm or rupture, myopia, detached lens, skeletal defects (long, thin arms, legs, toes, and fingers; pectus excavatum; scoliosis

81
Q

What is the defect and clinical relevance of scurvy?

A

Tropocollagen molecules cannot aggregate into fibrils due to decreased hydroxylation of proline caused by a deficiency in vitamin C

Gum ulceration and hemorrhages

82
Q

Where is the basal lamina found?

A

Epithelium
Kidney glomerulus
Muscle cells

83
Q

What are the components of the basal lamina?

A

Lamina lucida

Lamina densa

84
Q

What is the composition of lamina lucida?

A

Laminin, entactin, integrins, dystroglycans

85
Q

What is the composition of the lamina densa?

A

Type IV collagen, fibronectin, and perlecan

86
Q

What are the components of the basemen membrane?

A

Basal lamina + lamina fibronreticularis

87
Q

What is the composition of the lamina fibroreticularis?

A

Fibronectin and types I and III collagen

88
Q

What type of collagen serves to anchor the lamina densa and lamina fibroreticularis?

89
Q

How does membranous nephropathy come about?

A

Anitbodies attacking the basement membrane of the kidneys

90
Q

Observing a sample of kidney cells stained with silver methenamine through a light microcope, you see small, black projections coming off of the cells. What is your diagnosis?

A

Membranous nephropathy

Cell and tissue (45 decks)

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