Extracellular Matrix Disease

Question 1

Osteogenesis Imperfecta is associated with Type ____ Collagen defects

Answer 1

Type I, because it’s responsible for the bones, hence the osteogenesis

Question 2

Type IX collagen is associated with _____

Answer 2

Cartilage and Type II, found in chondrocytes

- Needed for joints

Question 3

Type I collagen is associated with _____

Answer 3

Bone, Skin, and Tendon

Question 4

Aggrecan is known as the other “9+2”, why?

Answer 4

Collagen IX and II link up with aggrecan in the chondrocyte matrix

Question 5

______ are surrounding by microfibrils (fibrillin) and if deficient, can cause Marfan Syndrome with mitral valve prolapse

Answer 5

Elastins

Question 6

Defects with Type IX collagen lead to ______

Answer 6

Arthritis

Question 7

Type II collagen is associated with __________

Answer 7

Cartialge, Ver. Discs, Notochord

(“The other 9+2”) associated with Type IX

Question 8

Mnemonic for Type IV collagen?

Answer 8

B4S4L L4MIN4

Question 9

Hyaluronic Acid, Chondroitin Sulfate, and Heparan sulfate are three examples of ______

Answer 9

GAGs

Question 10

The beta subunit of integrins binds to the cytoskeletal component known as _____. FAK then signals for cascades

Answer 10

Actin

Question 11

From top to bottom, Three layers of the basement membrane

Answer 11

1. Lamina lucida (clear)
2. Lamina densa (dark)
3. Lamina reticularis

Question 12

Glycosoaminoglycans have two main functions, what are they?

Answer 12

1. Resist Compressiong

2. Bring in Water

Question 13

Type III collagen is associated with ______

Answer 13

Skin, Blood vessels, and internal organs

Question 14

Mutations in collagen Type XVII cause blistiering Where in regards to the epithelium and Basement Membrane?

Answer 14

Between

Question 15

Most collagen lose their pro-extension-peptides, except for type ______

Answer 15

Type IV

Question 16

Ehler-Danlos syndrome is associated with Type ____ collagen defects

Answer 16

III, because that’s the one responsible for blood vessels and they tend to have a higher risk for aneurisms

Question 17

Aggrecan, Perlecan, and Syndecan are three examples of _____

Answer 17

Proteoglycans

Question 18

Mutations in collagen Type VII cause blistering Where in regards to the epitelium and basement membrane?

Answer 18

Below

Question 19

four main Integrin classes and their functions.

Answer 19

1. B1’s attach to ECM
2. B2’s bind with WBCs
3. B3’s bind Platelets
4. B4’s bind hemidesmosomes

Question 20

____ is a dimer that can bind proteoglycans, collagen, and integrin. “You may even think this triple function is sweet”

Answer 20

Fibro-Nectin

Question 21

Dr. Jones spoke of Aggrecan, what is its function?

Answer 21

Joint and mechanical support

Question 22

Two key distinguishing factors of Collagen Type VII?

Answer 22

1. Anchoring fibrils

2. Disulfide bonds

Question 23

Mnemonic for Syndecan?

Answer 23

“Cindeh Can Talk To Skeletons”

- Because Syndecan communicates with the cytoskeleton and binds to growth factors.

Question 24

The proteoglycan _____ uses heparan sulfate at the basal lamina to support the basement membrane. “Dance party at the basement”

Answer 24

PERLE-CAN

Question 25

3 Collagen Biosynthesis Steps

Answer 25

1. Make pro-alpha chain and hydroxylate 2. Glycosylate and make triple helix in ER/Golgi 3. Secrete into ECM and cleave so fibrils form

Question 26

What are the three repeating units of collagens?

Answer 26

Gly-X-Y | x and y are usually Proline and lysine

Question 27

Collagen Type XVII is a transmembrane collagen typicallyy associated with collagen type ____

Answer 27

7, to promote the anchoring.

Question 28

Four general sub-categories of Collagen?

Answer 28

1. Fibril-Forming 2. Fibril-Associated 3. Network-Forming 4. Trans-membrane