霍普金斯大学的科学家们已经发现了另一个原因,你应该听你的母亲时,她告诉你要多吃蔬菜。萝卜硫素,在西兰花中的前体形式高水平化学和相关的蔬菜(花椰菜、布鲁塞尔菜、等),有助于防止严重的水泡和皮肤破损所带来的难得的和潜在的致命性遗传疾病大疱性表皮松解症(EBS)。
研究人员用一种严重的EBS如此糟糕,他们三天内全部死亡和外用溶液含有萝卜硫素,显著提高治疗新生儿小鼠;经过四天以上的小鼠存活百分之85例,无气泡。这些研究结果出现在本周在线国家科学院学报。
The basis of EBS, notes study author Pierre Coulombe, Ph。D。, professor of biological chemistry, lies in two specific genes that make proteins known as keratins。 Normally, the keratins join together and form highly resilient fibers in the lower portion of skin, helping make it durable。 If either keratin is defective, they don’t mesh and the lower skin tissue becomes unusually fragile and gets damaged from the mildest mechanical stress - leading to blistering pain, a higher risk of infection, and in the most severe cases, death。
"Humans have around 54 distinct keratins, many of which are similar in structure and function," says Coulombe。 "We figured we might be able to exploit this similarity and dial up a replacement by triggering the activation of a suitable signaling pathway in skin。" He predicted that sulforaphane might stimulate the formation of a surrogate skin-strengthening keratin to stand in for the defective one。
The desire to learn more about sulforaphane led Coulombe and his co-workers to Paul Talalay, M。D。, professor of pharmacology who had previously identified sulforaphane as a cancer-preventive agent。 "It turns out that treatment with low doses of sulforaphane triggers the expression of selected keratin genes in skin," says Coulombe。 "So we began what evolved into a highly rewarding collaboration and found it does indeed work in a mouse model for EBS。"
"This is the first suggestion that we may be able to treat this terrible disease," adds Talalay, a co-author of this study。 "And we didn’t need to invent a new drug; sulforaphane is naturally found in our diet。"
The team will next test whether sulforaphane can stimulate the proper keratin protein in the appropriate subset of human skin cells - a vital matter for any future medical hopes。 Beyond that are issues of how effective a topical application would be on human skin, which is considerably thicker than mouse skin, as well as examining the long term effects of sulforaphane treatment。
"If we can clear these important hurdles, then sulforaphane can potentially be a tremendous therapeutic, with the added benefit of having anticancer properties," Coulombe says。 "And when you consider that the only current option for EBS is wrapping gauze around trauma-prone areas to minimize breakage, and otherwise avoiding infection and making sure blisters heal properly, then even a mild success would be a significant benefit for these patients。"
The research was funded by the National Institutes of Health, the March of Dimes Birth Defect Research Foundation, the American Institute for Cancer Research, and the Lewis B。 and Dorothy Cullman Foundation。
Authors on the paper are Michelle L。 Kerns, Daryle DePianto, Albena T。 Dinkova-Kostova, Talalay and Coulombe, all of Hopkins。
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