extracellular matrix

Question 1

what are the 3 components of the basement membrane?

Answer 1

lamina rara/lucida, lamina densa, lamina reticularis

Question 2

what is the difference between the basal lamina and the reticular lamina

Answer 2

the basal lamina is made of the lamina rara/lucida and lamina densa. the reitcular lamina is made of lamina reticularis

Question 3

what basement membrane look like under EM

Answer 3

like this

Question 4

what is the function of collagen compared to elastin in an ECM

Answer 4

collagen is for strngth and elastin is for resiliance

Question 5

what is unique to the alpha helix structure of collagen?

Answer 5

it goes Gly-X-Y-Gly so every third amino acid is glycine which allows it to fit into the helix because Gly is small and has no side chain

Question 6

what are some disease associated with collagen 1,2,3,7?

Answer 6

1. osteogenesis imperfecta
2. chondrodysplasia
3. ehler-danlos syndrome aneurism
4. epidermolysis

Question 7

what is the point of these propeptide ends?

Answer 7

there will not be incorporated into the final collagen. there are a part of the fibrins while in the ER because they keep the individual fibrins from interacting with each other and bonding until they are outside of the cell. this is when they assemble.

Question 8

what defect in type 7 collagen will cause blistering disease?

Answer 8

collagen 7 is an anchoring protein in the basal lamina. its individual fibrils assemble by attaching their c terminus. so a mutation in the c terminus will cause you to not be able to have collagen 7 so then there is no anchoring to basement membrane thus pemphigoid.

Question 9

what are some characteristics of marfan syndrome?

Answer 9

autosomal dominant

long arms, tall, leg,

Question 10

What are the GAGs and what they do?

Answer 10

GAGs are alternatinc acid sugar and amino acid. they bind water and resist comperession so they are found in joints and vitrous (eye)

Question 11

what are the list of GAGs you shoud know

Answer 11

Hyalyronic acid

chondroitin sulfate

heparan sulfate

Question 12

which collagen is found in cartilage

Answer 12

two

Question 13

which GAG does not bind protein

Answer 13

the one that doesnt have a sulfate which is also the famous one. Hyaluronic acid

Question 14

how do you make cartilage?? what are the components?

Answer 14

agrecan forms non coavalent bonds with hyaluronic acid to form cartilage

Question 15

syndican is unlike the other proteoglycans because

Answer 15

1. not formed entirely outside
2. its a transmembrane protein that interacts with the cytoskeleton
3. associates FGF with its receptor (cell adehsion)

Question 16

what are the components of the ECM (picture time)

Answer 16

a

Question 17

what is the relationship between focal adhesion molecules and integrins?

Answer 17

integrins bind actin and recruit focal adhesion molecules

Question 18

major classes of integrins (picture time)

Answer 18

remember you dont want B2 or B3 on all the time;.

Question 19

what is the disease caused by a defect in Beta 2 integrin

Answer 19

leukocyte adhesion deficiency. inability for WBC to bind endothelium

Question 20

what is the disease caused by B3 defect

Answer 20

Glanzann’s disease- inability to bind fibrinogen during clotting