Cell-cell/cell-matrix disorders Flashcards Preview

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Flashcards in Cell-cell/cell-matrix disorders Deck (11)
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1
Q

Pemphigus

A
  • a blistering disease

- caused by auto Ab’s to cadherins

2
Q

Glanzmann’s thrombasthenia

A

-inherited platelet integrin defect

3
Q

Metastatic cancer

A

-requires up regulation of matrix metalloproteinases

4
Q

menkes disease

A

-defect in collagen 1 cross-linking

5
Q

goodpasture syndrome

A

-autoimmune disease in which antibodies attack collagen 4 in the basement membrane

6
Q

alport syndrome

A

-due to mutations in collagen 4

7
Q

ehler-danlos syndrome

A

-defects in collagen synthesis and fibril assembly

8
Q

osteogenesis imperfecta

A

-mutations in collagen 1 gene

9
Q

scurvy

A

-collagen cross-linking defect due to vitamin C deficiency

10
Q

marfans syndrome

-mechanism detail

A
  • elastin cross linking defect usually due to defective fibrillin
  • elastin is a major component in the walls of large arteries and allows for expansion and compression during systole and diastole, respectively
  • this is especially true for the aorta
  • expansion is accomplished by the elastin molecules straightening out
  • fibrillin is responsible for not allowing the wall to stretch too far
  • if cross linking is defective than over time the wall will stretch and not reform, creating an aneurism
11
Q

angiogenesis

A

-the formation of new blood vessels requires MMP’s