ECM

Question 1

Functions of ECM

Answer 1

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

Question 2

2 Constituents of ECM

Answer 2

Group Substance and Fibers

Question 3

Group Substance

Answer 3

GAGs, proteoglycans, water, adhesive glycoproteins

Question 4

Fibers

Answer 4

collagen, reticular (type III) collagen, elastic

Question 5

Tissue repair with ECM

Answer 5

very vascular, edema, loose ECM

Question 6

How does ECM drive the cell cycle

Answer 6

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

Question 7

Excessive accumulation of GAGs leads to what

Answer 7

increase in water

Question 8

Structure of proteoglycans

Answer 8

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

Question 9

Syndecan

Answer 9

transmembrane proteoglycan. Co-receptor for FGF

Question 10

What happens when you inactivate gene for perlecan

Answer 10

results in defective skeletal development

Question 11

3 binding domains on multiadhesive glycoprotein

Answer 11

• Cell adhesion molecule (CAM)
• Collagen fibers
• Proteoglycans

Question 12

Binding domains of fibronectin

Answer 12

integrins, collagen, heparin, heparan fulfate, hyaluronic acid

Question 13

Location of fibronectin

Answer 13

connective tissue, blood plasma, embryonic tissue

Question 14

Binding domains of laminin

Answer 14

integrins, heparan sulfate, collagen IV, entractin

Question 15

Location of laminin

Answer 15

basal lamina

Question 16

Binding domains of entactin

Answer 16

laminin, integrins, type IV collagen

Question 17

location of entactin

Answer 17

basal lamina

Question 18

binding domains of tenscin

Answer 18

syndecans and fibrorecton

Question 19

Location of tenascin

Answer 19

embryonic tissue

Question 20

Binding domains of chondronectin

Answer 20

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

Question 21

Location of chondronectin

Answer 21

cartilage

Question 22

Binding domains of Ostonectin

Answer 22

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

Question 23

Location of osteonectin

Answer 23

bone

Question 24

What do antibodies to fibronectin do

Answer 24

Block branching morphogensis in developing mouse salivary glands

Question 25

Hydroxyproline

Answer 25

Used to calculate concentration of collagen

Question 26

Collagen can be turned over through what processes

Answer 26

Proteolytic degradation, Phagocytic degradation

Question 27

Function of Type I collagen

Answer 27

resistance to tension

Question 28

Function of Type II collagen

Answer 28

resistance to pressure

Question 29

Function of Type III collagen

Answer 29

maintain structure in expansion organs

Question 30

Function of Type IV collagen

Answer 30

support of delicate structures and filitration

Question 31

Function of Type V collagen

Answer 31

anchors basal lamina to lamina reticularis

Question 32

Tissue Distribution of Type I collagen

Answer 32

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

Question 33

Tissue Distribution of Type II collagen

Answer 33

hyaline and elastic cartilage

Question 34

Tissue Distribution of Type III collagen

Answer 34

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

Question 35

Tissue distribution of Type IV collagen

Answer 35

basal lamina

Question 36

Tissue distribution of Type V collagen

Answer 36

dermis

Question 37

Synthesizing cells of Type I collagen

Answer 37

fibroblasts, odontoblasts, cementoblasts, osteoblasts, chordroblasts

Question 38

Synthesizing cells of Type II collagen

Answer 38

chondroblasts

Question 39

Synthesizing cells of Type III collagen

Answer 39

smooth muscle cells, fibroblasts, reitcular cells

Question 40

Synthesizing cells of Type IV collagen

Answer 40

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

Question 41

Synthesizing cells of Type V collagen

Answer 41

epidermal cells

Question 42

Organization of Type I collagen

Answer 42

fibril-forming collagen leading to formation of fibers

Question 43

Organization of Type II collagen

Answer 43

fibril-forming collagen

Question 44

Organization of Type III collagen

Answer 44

fibril-forming collagen leading to formation of fibers

Question 45

Organization of Type IV collagen

Answer 45

network-forming collagen

Question 46

Organization of Type V collagen

Answer 46

anchoring collagen

Question 47

Composition of elastic fiber

Answer 47

proelastin, microfibril-associated glycoprotein, fibrillin

Question 48

Ehlers-Danlos Type IV

Answer 48

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

Question 49

Ehlers-Danlos Type VI

Answer 49

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

Question 50

Ehlers-Danlos Type VII

Answer 50

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

Question 51

Scurvy

Answer 51

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

Question 52

Osteogensis imperfecta

Answer 52

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

Question 53

Strickler syndrome

Answer 53

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

Question 54

Marfan Syndrome

Answer 54

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

Question 55

Lamina lucida contains

Answer 55

laminin, entractin, integrins, dystoglycans

Question 56

Lamina densa contains

Answer 56

type IV collagen, fibrorectin, perlecan

Question 57

Lamina fibroreticularis contains

Answer 57

fibrorectin, types I and III collagen

Question 58

Collagen VII

Answer 58

anchors lamina densa and lamina fibrorecularis