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UNDERSTANDING SCLERODERMA

ANCHOR LEAD: RESEARCHERS HAVE UNLOCKED SOME OF THE SECRETS OF THE CONNECTIVE TISSUE DISEASE SCLERODERMA, ELIZABETH TRACEY REPORTS

One in 5000 people develop scleroderma, a disease where both skin and internal organs may be affected by increased production of collagen and subsequent hardening.  Hal Dietz and colleagues at Johns Hopkins have studied the genetics of stiff skin syndrome, a rare disease, to help improve understanding of scleroderma.

DIETZ:  We found the gene that’s altered.  We learned that the defect prevents cells from being able to communicate with their outside environment.  The cells could no longer sense the amount of extracellular material and therefore had the signal that they needed to make more.  So cells would produce a lot of collagen which serves to make the tissues strong in the normal circumstance but serves to scar the tissue in scleroderma.    :30

Dietz says these insights provide a logical target for therapies that would interrupt the process of additional collagen production.  At Johns Hopkins, I’m Elizabeth Tracey.


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