Extracellular Matrix

Question 1

collagen I

Answer 1

fibril-forming in most connective tissues

skin, bone, tendon, cartilage, ligaments, connective tissue layers of muscle and nerves, capsules that surround internal organs, and the cornea

Question 2

collagen IV

Answer 2

network-forming in basal laminae

breaks in triple helical structure allow it to be more flexible

Question 3

collagen VII

Answer 3

anchoring fibrils / attachment of basal laminae to underlying connective tissue

crucial for functional integrity of epidermis-dermis junction in skin

Question 4

osteogenesis imperfecta

Answer 4

affects collagen I

weak bones, fracture easily

Question 5

Alport syndrome

Answer 5

affects collagen IV

hearing loss, ocular lesions, and progressive nephritis

Question 6

DEB (dystrophic epidermolysis bullosa)

Answer 6

affects collagen VII

epidermis-dermis junctions is compromise; skin blisters form during minor friction, heat, or minor injury

Question 7

Structure of collagen

Answer 7

triple helical structure

Question 8

function of collagen

Answer 8

makes up fibrils of connective tissue; forms networks, anchoring fibrils, and forms transmembrane domains to hold membranes together

Question 9

structure of fibrillin

Answer 9

forms microfibrils and associates with elastic fibers in the ECM

Question 10

function of fibrillin

Answer 10

provides scaffold for tissues

Question 11

structure of proteoglycans

Answer 11

lot of sugar attached to little protein

Question 12

function of proteoglycans

Answer 12

bind growth factors; also found in kidney and joints (arthritis - proteoglycan breakdown)