cell matrix

Question 1

connective tissue matrix components (4)

Answer 1

elastic fiber
proteoglycan
hyaluronan
Type I collagen

Question 2

Basement membrane is the same as the

Answer 2

Basal Lamina

Question 3

thin protein membrane based structure

Answer 3

Basement membrane

Question 4

sugars on the proteoglycan create

Answer 4

a strong barrier

Question 5

genetic defect causes deficinecy in copper utilization and thus faulty collagen crosslinking

Answer 5

Menke disease

Question 6

Genetic defect in which Type IV collagen is absent or non-functional.

Answer 6

Alport syndrom

Question 7

Collagen IV commonly found in

Answer 7

Basement membrane

Question 8

second most common inherited PKD

Answer 8

Alport syndrome

Question 9

autoimmunie disease in which antibodies to Type IV collagen destroy the basement membrane in the lung and kidneys

Answer 9

Goodpasture syndrome

Question 10

20 kd fragment of Collagen XVIII

Answer 10

blood vessel-specific endostatin

Question 11

endostatin is cleaved from

Answer 11

Collagen XVIII by an MMP

Question 12

used in 4 million patients; endostatin, angiostatin and anti-VEGF antibody

Answer 12

angiogenesis

Question 13

endostatin specificity

Answer 13

immature vascular endothelial cells

Question 14

Endostatin likely mechanism

Answer 14

induces apoptosis in EC

Question 15

ATP7A gene defect

Answer 15

menke

Question 16

proteoglycan major function

Answer 16

provide selectively permeable filters in kidney and lung

Question 17

more protein than carbohydrates and N-linked

Answer 17

glycoproteins

Question 18

o-linked, huge amount of carbohydrates vs protein

Answer 18

proteogylcans

Question 19

chondrodysplasias (2) causes

Answer 19

(1) defective chondroitin sulfate proteoglycans

(2) defective collagen II

Question 20

secreted by cell and remain tightly bounds to cell surface receptors, including integrins

Answer 20

matricellular proteins

Question 21

what are the major classes of matricellular proteins

Answer 21

• fibronectins
• laminins
• CCN proteins

Question 22

Matricellular proteins often act as _______ to help regulate ______ and ___________ ________ for proliferation, motility and differentiation

Answer 22

Matricellular proteins often act as co-receptors to help regulate adhesion and signal transduction pathways for proliferation, motility and differentations

Question 23

epithelial cell matrix (basement membrane)

Answer 23

laminin

Question 24

another common adhesive protein that binds to integrins, heparan sulfates and collagens is

Answer 24

laminin

Question 25

laminin is a major component of

Answer 25

basement membranes

Question 26

key point of integrins

Answer 26

they are everywhere

Question 27

an inherited platelet integrin defect

Answer 27

Glanzmann's

Question 28

elastin fibers are critical in

Answer 28

large arteries

Question 29

elastin fibers are critical in

Answer 29

large arteries

Question 30

mechanism of elastin-based stretch is through

Answer 30

fibrillin

Question 31

contracted "elastin"

Answer 31

low energy, high entropy

Question 32

defective fibrillin fails to cross-link elastin

Answer 32

marfan's syndrome

Question 33

required for angiogenesis

Answer 33

MMPs

Question 34

cadherins are important in

Answer 34

junctions

Question 35

an inflammatory disease involving complex cell-cell and cell-matrix interactions

Answer 35

atherosclerosis

Question 36

atherosclerosis starts with

Answer 36

endothelial dysfunction

Question 37

fatty streak formation

Answer 37

- foam-cell formation - T-cell activation - Smooth muscle cell migration - adherence and aggregation of platelets - adherence and entry of leukocytes

Question 38

advanced atherosclerosis

Answer 38

- macrophage accumulation - formation of necrotic core - fibrous -cap formation

Question 39

unstable atherosclerotic plaques

Answer 39

- how much blood flow is present | - rapidly forming plaque and rupture

Question 40

what control leukocyte adhesion to endothelium

Answer 40

CAMs and Matricellular Proteins

Question 41

leukocytes _____ along endothelium via weak "on-off" interactions of ______ on the leukocyte membrane with ____ on the endothelium

Answer 41

leukocytes roll along the endothelium via weak "on-off" interactions of carbohydrates on the leukocyte membrane with P-selectin on the endothelium

Question 42

P-selectin

Answer 42

matricellular protein

Question 43

Leukocytes _____ and ______ into inflamed areas due to PAF-activated _____ on the leukocyte binding strongly to _______ on the endothelium

Answer 43

Leukocytes stick and extravasate into inflamed areas due to PAF- activated integrins on the leukocyte binding strongly to ICAM-1 on the endothelium

Question 44

ICAM-1

Answer 44

cell adhesion molecule

Question 45

ATP7A is a

Answer 45

P-type ATPase

Question 46

which of the collagen-based diseases be managed by corticosteroids and other immunosuppressants

Answer 46

Goodpasture

Question 47

other major functions of proteoglycans (3)

Answer 47

(1) regulate hydration state of tissues, especially cartilage (2) provide resistance to impact in cartilage (3) Act as reservoirs for growth factors and cytokines