ECM Flashcards

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1
Q

Functions of ECM

A

Structure, Defense and protection, Nutrition, Diffusion of gases, molecules and ions, Cell growth and survival, Cell migration, Lubrication

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2
Q

2 Constituents of ECM

A

Group Substance and Fibers

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3
Q

Group Substance

A

GAGs, proteoglycans, water, adhesive glycoproteins

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4
Q

Fibers

A

collagen, reticular (type III) collagen, elastic

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5
Q

Tissue repair with ECM

A

very vascular, edema, loose ECM

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6
Q

How does ECM drive the cell cycle

A

Focal adhesions of cell to ECM regulate cell division, growth and survival

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7
Q

Excessive accumulation of GAGs leads to what

A

increase in water

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8
Q

Structure of proteoglycans

A

Core protein is the backbone. Core protein is attached to link protein on hyaluronan. GAGs attach to the core protein and look like bristles

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9
Q

Syndecan

A

transmembrane proteoglycan. Co-receptor for FGF

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10
Q

What happens when you inactivate gene for perlecan

A

results in defective skeletal development

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11
Q

3 binding domains on multiadhesive glycoprotein

A
  • Cell adhesion molecule (CAM)
  • Collagen fibers
  • Proteoglycans
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12
Q

Binding domains of fibronectin

A

integrins, collagen, heparin, heparan fulfate, hyaluronic acid

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13
Q

Location of fibronectin

A

connective tissue, blood plasma, embryonic tissue

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14
Q

Binding domains of laminin

A

integrins, heparan sulfate, collagen IV, entractin

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15
Q

Location of laminin

A

basal lamina

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16
Q

Binding domains of entactin

A

laminin, integrins, type IV collagen

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17
Q

location of entactin

A

basal lamina

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18
Q

binding domains of tenscin

A

syndecans and fibrorecton

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19
Q

Location of tenascin

A

embryonic tissue

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20
Q

Binding domains of chondronectin

A

Type II collagen, chondroitin sulfates, hyaluronic acid, integrins of chondrocytes

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21
Q

Location of chondronectin

A

cartilage

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22
Q

Binding domains of Ostonectin

A

Type I collagen, proteoglycans and integrins of bone cells (osteocytes, osteoblasts)

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23
Q

Location of osteonectin

A

bone

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24
Q

What do antibodies to fibronectin do

A

Block branching morphogensis in developing mouse salivary glands

25
Q

Hydroxyproline

A

Used to calculate concentration of collagen

26
Q

Collagen can be turned over through what processes

A

Proteolytic degradation, Phagocytic degradation

27
Q

Function of Type I collagen

A

resistance to tension

28
Q

Function of Type II collagen

A

resistance to pressure

29
Q

Function of Type III collagen

A

maintain structure in expansion organs

30
Q

Function of Type IV collagen

A

support of delicate structures and filitration

31
Q

Function of Type V collagen

A

anchors basal lamina to lamina reticularis

32
Q

Tissue Distribution of Type I collagen

A

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

33
Q

Tissue Distribution of Type II collagen

A

hyaline and elastic cartilage

34
Q

Tissue Distribution of Type III collagen

A

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

35
Q

Tissue distribution of Type IV collagen

A

basal lamina

36
Q

Tissue distribution of Type V collagen

A

dermis

37
Q

Synthesizing cells of Type I collagen

A

fibroblasts, odontoblasts, cementoblasts, osteoblasts, chordroblasts

38
Q

Synthesizing cells of Type II collagen

A

chondroblasts

39
Q

Synthesizing cells of Type III collagen

A

smooth muscle cells, fibroblasts, reitcular cells

40
Q

Synthesizing cells of Type IV collagen

A

endothial cells, epithial cells, Schwann cells, smooth muscle cells

41
Q

Synthesizing cells of Type V collagen

A

epidermal cells

42
Q

Organization of Type I collagen

A

fibril-forming collagen leading to formation of fibers

43
Q

Organization of Type II collagen

A

fibril-forming collagen

44
Q

Organization of Type III collagen

A

fibril-forming collagen leading to formation of fibers

45
Q

Organization of Type IV collagen

A

network-forming collagen

46
Q

Organization of Type V collagen

A

anchoring collagen

47
Q

Composition of elastic fiber

A

proelastin, microfibril-associated glycoprotein, fibrillin

48
Q

Ehlers-Danlos Type IV

A

Caused by mutation in COLI3A1 gene encoding type III collagen. Leads to varicose veins, aortic rupture, intenstinal rupture

49
Q

Ehlers-Danlos Type VI

A

Caused by defective hydroxylation of lysine thereby destabilizing the strenth of collagen. Leads to hyperelasticity of skin, rupture of eyeball

50
Q

Ehlers-Danlos Type VII

A

Caused by mutations in the COL1A1 and COL1A2 genes encoding type I collagen. Leads to joint dislocation and hypermobility of joints

51
Q

Scurvy

A

Caused by tropocollagen molecules that cannot aggregate into fibrils due to decreased hydroxylation of proline caused by deficency in vitamin C. Results in gum ulceration and hemorrhages

52
Q

Osteogensis imperfecta

A

Caused by mutations in COL1A1 leading to reduction in synthesis of type I collagen. Leads to spontaneous fractures, cardiac insufficiency

53
Q

Strickler syndrome

A

Caused by mutation in COL2A1 gene encoding type II collagen. Leads to myopia, hypoplasia of mandible, arthritis

54
Q

Marfan Syndrome

A

Caused by mutation of fibrillin I gene located on chromosome 15 - defective synthesis of elastic fibers. Leads to aortic aneurysm or rupture, myopia, detached lens, and skeletal defects

55
Q

Lamina lucida contains

A

laminin, entractin, integrins, dystoglycans

56
Q

Lamina densa contains

A

type IV collagen, fibrorectin, perlecan

57
Q

Lamina fibroreticularis contains

A

fibrorectin, types I and III collagen

58
Q

Collagen VII

A

anchors lamina densa and lamina fibrorecularis