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Flashcards in Extracellular Matrix Disease Deck (28)
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1
Q

Osteogenesis Imperfecta is associated with Type ____ Collagen defects

A

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

2
Q

Type IX collagen is associated with _____

A

Cartilage and Type II, found in chondrocytes

- Needed for joints

3
Q

Type I collagen is associated with _____

A

Bone, Skin, and Tendon

4
Q

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

A

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

5
Q

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

A

Elastins

6
Q

Defects with Type IX collagen lead to ______

A

Arthritis

7
Q

Type II collagen is associated with __________

A

Cartialge, Ver. Discs, Notochord

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

8
Q

Mnemonic for Type IV collagen?

A

B4S4L L4MIN4

9
Q

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

A

GAGs

10
Q

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

A

Actin

11
Q

From top to bottom, Three layers of the basement membrane

A
  1. Lamina lucida (clear)
  2. Lamina densa (dark)
  3. Lamina reticularis
12
Q

Glycosoaminoglycans have two main functions, what are they?

A
  1. Resist Compressiong

2. Bring in Water

13
Q

Type III collagen is associated with ______

A

Skin, Blood vessels, and internal organs

14
Q

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

A

Between

15
Q

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

A

Type IV

16
Q

Ehler-Danlos syndrome is associated with Type ____ collagen defects

A

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

17
Q

Aggrecan, Perlecan, and Syndecan are three examples of _____

A

Proteoglycans

18
Q

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

A

Below

19
Q

four main Integrin classes and their functions.

A
  1. B1’s attach to ECM
  2. B2’s bind with WBCs
  3. B3’s bind Platelets
  4. B4’s bind hemidesmosomes
20
Q

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

A

Fibro-Nectin

21
Q

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

A

Joint and mechanical support

22
Q

Two key distinguishing factors of Collagen Type VII?

A
  1. Anchoring fibrils

2. Disulfide bonds

23
Q

Mnemonic for Syndecan?

A

“Cindeh Can Talk To Skeletons”

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

24
Q

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

A

PERLE-CAN

25
Q

3 Collagen Biosynthesis Steps

A
  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
26
Q

What are the three repeating units of collagens?

A

Gly-X-Y

x and y are usually Proline and lysine

27
Q

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

A

7, to promote the anchoring.

28
Q

Four general sub-categories of Collagen?

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