Washington: Scientists claim to havefinally discovered the causes for the stiff skin syndrome, abreakthrough which may pave the way for new treatments.A team at Johns Hopkins University has studied thegenetics of stiff skin syndrome to learn about scleroderma,a condition that leads to hardening of the skin as well asother debilitating and often life-threatening problems, the`Science Translational Medicine` journal reported.
So Dietz`s team examined patients with stiff skinsyndrome and found them to have excessive amounts offibrillin-1 in the skin. The researchers then sequenced the fibrillin-1 genein these same patients and found all the stiff skin syndromemutations clustered in a single region of the fibrillin-1protein known to interact with neighboring cells. Further examination showed that these mutationsprevent fibrillin-1 from interacting with neighboring cellsand lead to increased amounts and activity of TGFbeta, whichcauses excessive collagen outside cells. "It appears that fibriillin-1 helps to inform cellsabout the quality of their surroundings and also provides amechanism -- by concentrating TGFbeta -- to induce extracellular matrix production if the cell senses a deficiency. "A breakdown in signaling coupled with excessivefibrillin-1 and TGFbeta leads to a perfect storm for skinfibrosis in stiff skin syndrome," Dietz said. PTI
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