ECM/BM Flashcards

0
Q

Soluble components of ECM

A

proteoglycans- made up of GAG (ex. hyaluronan)

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1
Q

Cells primarily responsible for making ECM?

A

fibroblasts

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2
Q

2 examples of proteoglycans

A

Aggrecan (LARGE) - in cartilage)
Decorin (large)- one GAG chain

(soluble ECM)

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3
Q

Hyaluronan?

A

type of GAG (soluble ECM)

abundant in joint fluid

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4
Q

Insoluble components of ECM

A

collagen
elastin
fibronectin
laminin

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5
Q

Collagen:

  • structure
  • degradation
A

alpha triple helix; glycosylated

cleaved by MMP-1 (TIMP inhibits MMP-1)

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6
Q

Elastin precursor?

A

Tropoelastin (soluble)

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7
Q

Fibronectin:

  • structure
  • primary component of what?
  • associated with what?
A
  • dimer joined by disulfide bonds (will bind collagen and heparin)
  • delivered to site of injury to form blood clot
  • cancer, fibrosis
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8
Q

Laminin:

  • structure
  • role in WH?
A

long protein, 3 long polypeptide chains

laminin 322 deposited in WH

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9
Q

MMP-1?

A

cleaves collagen; inhibited by TIMP

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10
Q

Aggrecan?

A

LARGE proteoglycan (soluble ECM)

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11
Q

Is elastin glycosylated? is collagen?

A

elastin is nnot glysylated; collagen is

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12
Q

Disorders ECM

A

Marfan syndrome- glycoprotein scaffold for elastin
Ehlers-danlos syndrome (EDS)- collagen
Osteogenesis imperfecta- collagen
Venous malformation

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13
Q

Decorin?

A

Proteoglycan (soluble ECM)

one GAG chain

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14
Q

Ehlers danlos syndrome

  • gene?
  • defect?
  • clinical signs
A

COL5A/COL3A
collagen synthesis defect
skin fragility, hyperextendability

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15
Q

BM: occluding junction?

A
cell-cell 
zonula occludens (tight junction)
defines apical domain
16
Q

Anchoring junctions? (4)

A

Cell-cell
-zonula adhereins (actin cytoskeleton - PM)
-macula adherens (desmosome) (intermediate filament - PM)
Cell-ECM
-focal adhsions (actin cytoskeleton - ECM; transmit signal out to in)
-hemidesmosome (intermediate filament-ECM)

17
Q

Communicating junctions?

A

cell-cell

gap junction - pass ions

18
Q

Marfan syndrome

  • gene
  • protein involved
A

FBN-1
fibrillin-1 (glycoprotein that provides scaffold for elastin)
long limbs