[ad_1]
Children with a serious skin disorder called butterfly syndrome develop severe, chronic blisters. Fibrosis, thickening and scarring of connective tissues, is a major complication of the disease. Fibrosis can not only lead to club-shaped appendages where the skin grows above the fingers or toes, but Dr. Andrew South of Jefferson, an badociate professor in the Department of Dermatology and Skin Biology, has shown a Aggressive and often fatal skin cancer. Now, Dr. South and his colleagues have pointed out the development of fibrosis in patients with butterfly syndrome. The discovery suggests a potential treatment for the debilitating complication.
Scientists suspected a protein called thrombospondin-1 (TSP1) to be involved in fibrosis. In previous research, Dr. South and colleagues found that skin cells from butterfly syndrome patients had more TSP1 than the skin cells of healthy individuals. In the new study, researchers have shown that TSP1 binds to a protein that helps to maintain layers of skin called collagen 7 (C7) in the skin cells of healthy individuals. But C7 is lacking in patients with butterfly syndrome.
"We are showing for the first time in human cells that TSP1 binds to collagen 7 when it is present," says Dr. South, who published the results online on Jan. 23. Journal of Investigative Dermatology. As children with butterfly syndrome do not have much or no collagen 7, TSP1 is able to bind to a different molecule called transforming growth factor-β (TGFβ). TGFβ is a well-known signaling molecule that, when activated, triggers a cascade of molecular signals leading to fibrosis.
"Our data indicates that it is the leading factor of fibrosis in these patients," says Dr. South, also a researcher at the Sidney Kimmel Cancer Center – Jefferson Health.
The researchers then showed that a molecule preventing TSP1 from activating TGFβ reduces fibrosis in a tissue engineering model of the symptom. In addition to this potential therapy, researchers are currently examining nearly 1,500 FDA-approved molecules for other treatments.
"Now that we know that one of the main activators of fibrosis is the TSP1, we are trying to determine if it is possible to change the use of any of these drugs to treat fibrosis in patients with butterfly syndrome, "says Dr. South.
Source of the story:
Material provided by Thomas Jefferson University. Note: Content can be changed for style and length.
Source link
