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Extracellular Matrix
Seasholtz
9
Medical
Professional
09/15/2012

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Term
Roles of ECM
Definition
-Stabilizing scaffold
-influences survival, development, proliferation, migration, function
Term
ECM Production
Definition
fibroblasts
osteoblasts
chondrocytes
Term
Collagen structure
Definition
Gly-X-Y
X: proline
Y: hydroxyproline/hydroxylysine
left handed helix, 3 AAs/turn
Glycine in interior
Term
Collagen Types
Definition
-Fibril forming, interstitial (I, II, III, V, XI)
-FACIT (IX, XII, XIV)
-Network forming (IV)
-Anchoring filaments (VII)
Term
Fibrillar Collagens
Definition
Type I: bone, tendon, dermis, cornea, dentin. Osteogenesis imperfecta
Type II: cartilage, vitreous humor. Stickler syndrome
Type III: reticular fibers of most tissues (lung, liver, dermis, vessel walls). Vascular E-D Syndrome
Type V: dermis, interstitial tissues. Classical E-D syndrome
Term
Collagen synthesis
Definition
1. pro-alpha chain synthesis
2. hydroxylation of prolines and lysines (prolyl hydroxylase requires vitamin C)
3. glycosylation of hydroxylysines
4. self-assembly of pro-alpha chains
5. procollagen triple-helix formation
6. secretion
7. cleavage of propeptides (procollagen --> tropocollagen) (procollagen N-proteinase and procollagen C-proteinase)
8. self-assembly into fibril (driven by insolubility of tropocollagen)
9. aggregation of collagen fibrils to form a collagen fiber
Term
Collagen crosslinks
Definition
1. lysyl oxidase (Cu requiring enzyme): acts on lysines and hydroxylysines, forms covalent bonds
2. most form in non-helical segments
3. can be intramolecular/intermolecular
Term
FACIT Collagens IX, XII, XIV
Definition
Fibril associated collagens with interrupted triple helices
-link fibrils to one another and other ECM macromolecules
-globular N-terminus of IX binds to proteoglycans in ECM
-helical domains interact w collagen type II (esp. important in cartilage)
Term
Network Forming Collagen (IV)
Definition
-Major type in basal lamina
-flexible structure: interrupted triple helix, flexible sheet-like multilayered network
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