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A tribute to Professor Christian de Duve on his 90th birthday.

机译:向克里斯蒂安·德·杜夫教授诞辰90周年致敬。

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As its name implies, the Journal of Cellular and Molecular Medicine is an appropriate stage for a tribute to Professor C. de Duve on his 90th birthday. Indeed, the work of Christian de Duve led to an explosion in cell biology, with the discovery of lyso-somes and peroxisomes and of their function. From the deciphering of the physiopathology of lysosomes followed the first molecular explanation of an intracel-lular genetic disorder, Pompe's glycogenosis, the prototype of lysosomal storage diseases. In turn, this basic knowledge paved the way to rational molecular medicine, with the first effective cure of another inborn lysosomal disorder, Gaucher's disease, by replacement therapy with recombinant glucocere-brosidase, now a current practice. How did this story develop and what does the example of de Duve tell us?
机译:顾名思义,《细胞与分子医学杂志》是向C. de Duve教授90岁生日致敬的合适舞台。确实,克里斯汀·德·杜夫(Christian de Duve)的工作导致了细胞生物学的爆炸式增长,发现了溶酶体和过氧化物酶体及其功能。从溶酶体的生理病理学破译开始,对细胞内遗传疾病,庞培糖原病(溶酶体贮积病的原型)进行了第一个分子解释。反过来,这一基础知识又为合理的分子医学铺平了道路,该方法通过重组葡糖纤溶酶替代疗法(目前已成为一种实践),首次有效治愈了另一种先天性溶酶体疾病,即高雪氏病。这个故事是如何发展的,德杜夫的例子告诉我们什么?

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