What is the role of serum tissue inhibitor of metalloproteinase-1 (TIMP-1) in the pathophysiology of CREST syndrome?

Updated: Oct 05, 2020
  • Author: Jeanie C Yoon, MD; Chief Editor: Dirk M Elston, MD  more...
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Serum tissue inhibitor of metalloproteinase-1 (TIMP-1) levels are elevated in scleroderma patients compared with normal controls. This may allow progressive fibrosis to result because of a relative lack of collagenase activity. TIMP-1 may behave as an autocrine growth factor in the fibrotic process of scleroderma. [20] Recently, the protease nexin-1 gene (PN1) has been found to be overexpressed in systemic sclerosis fibroblasts. PN1 plays an important role in the regulation of cell growth, differentiation, and cell death by modulating proteolytic activity; in vitro evidence suggests it inhibits metalloproteinase activation. [21]

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