Collagen

  • Family of fibrous proteins, 20+ genetically distinct types identified; provides structural stability and accounts for 70–80% dry weight of dermis; major dermal constituent
  • Composed of three chains combined into a triple helix configuration; contains Gly-x-y repeats (glycine always third residue, x frequently proline, y often hydroxylysine or hydroxyproline)
  • Collagen degraded by interstitial collagenases (metalloproteinases or MMPs)
  • Collagen synthesis stimulated by retinoic acid
  • Collagen synthesis inhibited by: IL-1 (↑ MMP expression), glucocorticoids, IFNγ, TNFα, D-penicillamine, UV irradiation
   
 
Table 1-5 Types of Collagen
Collagen
Location
Associated Diseases
I
 
Dermis, bone, ligament/tendon
 
Ehers-Danlos syndrome, arthrochalasia (EDS type VII), osteogenesis imperfecta
II
Vitreous humor, cartilage
III
 
Fetal skin, blood vessels
 
EDS vascular (type IV)
IV
Basement membrane
Alport and Goodpasture syndrome
V
 
Ubiquitous
 
EDS classic (type I/II)
VI
Aorta, placenta
Congenital muscular dystrophy
VII
 
Anchoring fibrils (BMZ)
 
Dystrophic EB (DEB)
VIII
Cornea (Descemet’s membrane: basement membrane between corneal proper substance and endothelial layer)
Corneal dystrophy
IX-XII
 
Cartilage
 
-
XV–XVI
Placenta
-
XVII (BPAG2)
 
Hemidesmosome
 
Junctional EB (JEB)