What is the pathogenesis of skin fibroblasts in CREST syndrome?

Updated: Oct 05, 2020
  • Author: Jeanie C Yoon, MD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Skin fibroblasts in patients with scleroderma act as if they are persistently activated. Higher levels of COL1A2 mRNA (gene encoding alpha-2 chain of type I procollagen) are found in the dermis of scleroderma patients compared with patients without scleroderma, and down-regulation of fibroblast collagen synthesis by collagen amino-terminal peptides is impaired.


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