ECM/BM Flashcards
Soluble components of ECM
proteoglycans- made up of GAG (ex. hyaluronan)
Cells primarily responsible for making ECM?
fibroblasts
2 examples of proteoglycans
Aggrecan (LARGE) - in cartilage)
Decorin (large)- one GAG chain
(soluble ECM)
Hyaluronan?
type of GAG (soluble ECM)
abundant in joint fluid
Insoluble components of ECM
collagen
elastin
fibronectin
laminin
Collagen:
- structure
- degradation
alpha triple helix; glycosylated
cleaved by MMP-1 (TIMP inhibits MMP-1)
Elastin precursor?
Tropoelastin (soluble)
Fibronectin:
- structure
- primary component of what?
- associated with what?
- dimer joined by disulfide bonds (will bind collagen and heparin)
- delivered to site of injury to form blood clot
- cancer, fibrosis
Laminin:
- structure
- role in WH?
long protein, 3 long polypeptide chains
laminin 322 deposited in WH
MMP-1?
cleaves collagen; inhibited by TIMP
Aggrecan?
LARGE proteoglycan (soluble ECM)
Is elastin glycosylated? is collagen?
elastin is nnot glysylated; collagen is
Disorders ECM
Marfan syndrome- glycoprotein scaffold for elastin
Ehlers-danlos syndrome (EDS)- collagen
Osteogenesis imperfecta- collagen
Venous malformation
Decorin?
Proteoglycan (soluble ECM)
one GAG chain
Ehlers danlos syndrome
- gene?
- defect?
- clinical signs
COL5A/COL3A
collagen synthesis defect
skin fragility, hyperextendability