ECM

Question 1

basic elements of the ECM

Answer 1

ground substance
fibers
granulation tissue

Question 2

syndecans

Answer 2

proteoglycan
core protein mcl is embedded in the cell membrane as a transmembrane protein
serves as a receptor for fibroblast growth factor

Question 3

perlecans

Answer 3

proteoglycan in basement membrane
functions: cell adhesion, proliferation, differentiation, glomerular filtration, development, and growth factor binding
enhanced in some tumors

Question 4

adhesive glycoproteins have 3 binding sites

Answer 4

• integrins
• collagen fibers
• proteoglycans

Question 5

fibronectin

Answer 5

binds to inegrins, collage, heparin, heparan sulfate, and hyaluronic acid
-located in connective tissue, blood plasma, and embryonic tissue

Question 6

laminin

Answer 6

binds to integrins, heparan sulfate, collagen IV, and entactin
located in basal lamina

Question 7

entactin

Answer 7

binds to laminin and type IV collagen

located in basal lamina

Question 8

tenascin

Answer 8

binds to syndecans and fibronectin

located in embryonic tissue

Question 9

chondronectin

Answer 9

binds to collagen II, chondritin sulfates, hyaluronic acid, and integrins of chrondrocytes
located in cartilage

Question 10

osteonectin

Answer 10

binds to collagen I, proteoglycans, integrins of bone cells

located in bone

Question 11

collagen I

Answer 11

tissue: dermis, tendon, bone, dentin, cementum, fibrocartilage, organ capsules
cells: fibroblasts, ondontoblasts, cementoblasts, osteoblasts, chrondroblasts
function- resistance to tension, fibril forming leading to fibers

Question 12

collagen II

Answer 12

tissue: hyaline and elastic cartilage
cells: chrondroblasts
function: resistance to pressure, fibril forming

Question 13

collagen III

Answer 13

tissue: spleen, liver, lymph nodes, smooth muscle, skin, lung
cells: smooth muscle cells, fibroblasts, reticular cells
function: maintains structure, fibril forming to fibers

Question 14

collagen IV

Answer 14

tissue: basal lamina
cells: endothelial cells, epithelial cells, schwann cells, smooth muscle cells
function: support delicate structures, network forming

Question 15

collagen VII

Answer 15

tissue: dermis
cells: epidermal cells
function: anchors lamina densa to the lamina reticularis, anchoring collagen

Question 16

ehlers danlos IV

Answer 16

COL3A1 mutation in type III collagen

varicose veins, aortic rupture, intestinal rupture

Question 17

ehlers danlos VI

Answer 17

defective hydroxylation of lysine, destabilizing collagen strength
hyperelasticity of skin, rupture eyeball

Question 18

ehlers danlos VII

Answer 18

COL1A1 & COL1A2 mutations encoding collagen I

joint dislocation and hypermobility of joints

Question 19

scurvy

Answer 19

decreased hydroxylation of proline, deficiency in vit c

gum ulceration and hemorrhages

Question 20

osteogenesis imperfecta

Answer 20

COL1A1 mutation reduced synthesis collagen I

spontaneous fractures and cardiac insufficiency

Question 21

strickler syndrome

Answer 21

COL2A1 mutation for collagen II

myopia, hypoplasia of mandible, arthritis

Question 22

marfan syndrome

Answer 22

mutation on fibrillin 1 gene on chromosome 15

aortic aneurysm, myopia, detached lens, skeletal defects