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Collagens are responsible for the tensile strength of the dermis and are composed of triple helix a- chains. What is the amino acid sequence for collagen?
Gly-x-y
high numbers of proline and hydroxyproline in the X and Y positions
hydrogen bonds between hydroxyproline give collagen strength
What percentage of collagen makes up the dry weight of the dermis?
75%
Most collagens in the skin are made by dermal fibroblasts.
EXCEPT!
Epidermal keratinocytes and fibroblasts systhesize...
7 & 17 are synthesized by epidermal keratinocytes and fibroblasts
Endothelial cells synthesize...
8 & 18 are synthesized by endothelial cells
Hightlights of collagen synthesis?
1. pre-pro a chains synthesized
2. hydroxylation/ glycosylation
3. disulfide bonds between hydroxyl groups
4. triple helix formation of procollagen
5. cleavage of N and C terminus
6. assembly into fibrils with covalent crosslinks
In collagen synthesis, ascorbic acid is a co factor for...
Vit C is required to hydroxylate proline
all the pro's take Vit C!
In collagen synthesis, copper is a co factor for...
Copper is required to hydroxylate lysine
What disorder is associated with a defect in lysyl hydroxylase?
EDS- kyphoscoliosis (PLOD gene)
What disorder is associated with a defect in Procollagen N- proteinase gene ? What is this gene called?
ADAMTS-2
EDS- DERMATOSPARAXIS
Adam is a little sprite (fairy?)
What disorder is associated with a mutation in the amino terminal propeptide cleavage sites of COL1A1 or COL1A2?
EDS- arthrochalasia
arthro <-> ortho <-> bones, type I collagen
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