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引用次数: 0
摘要
吉莉安-巴特勒-布朗于 1978 年开始在巴斯德研究所弗朗索瓦-格罗斯实验室从事肌肉研究。她研究了人类和啮齿动物发育过程中不同肌球蛋白同工酶的表达谱。1982年,文森特-穆利(Vincent Mouly)加入该实验室攻读博士学位,并确定了在鸟类和人类发育过程中出现的不同肌细胞群。他们共同证明了前体细胞增殖限制对人类骨骼肌再生能力的影响,他们的研究小组开发了评估体外骨骼肌再生潜力的模型,测量端粒侵蚀,并确定了肌肉祖细胞增殖停滞的应激途径。与弗朗索瓦-格罗斯(François Gros)和 W. E. 赖特(W. E. Wright)共同发起的这项研究成功地建立了一个生产人类永生肌肉细胞系的平台。通过向免疫缺陷小鼠的肌肉注射,对人类肌肉细胞的体内再生潜力进行了评估。他们的研究小组与让-拉考-圣吉利(Jean Lacau St-Guily)教授和索菲-佩里(Sophie Perié)教授的临床团队合作,成功完成了治疗眼咽肌营养不良症的自体肌细胞移植临床试验。这一共同的科学事业之所以能够取得成功,要归功于弗朗索瓦-格罗斯(François Gros)始终给予的宝贵而仁慈的支持。
Gillian Butler-Browne began working on muscle at the Institut Pasteur in the laboratory of François Gros in 1978. She characterized the expression profile of different myosin isoforms during both human and rodent development. Vincent Mouly joined this laboratory for his PhD in 1982, and defined the different populations of myoblasts appearing during development in birds and then in humans. Together, they demonstrated the impact of the limit in proliferation of the precursor cells on the regenerative capacity of human skeletal muscle, and their group developed models to evaluate the regenerative potential of skeletal muscle in vitro, measuring the telomeric erosion, and identified the involvement of a stress pathway in the proliferative arrest of muscle progenitors. A platform to produce human immortalized muscle cell lines was the successful result of this research, initiated with François Gros and W. E. Wright. The in vivo regenerative potential of human muscle cells was evaluated by injection into muscles of immunodeficient mice. Their group in collaboration with the clinical team of Professor Jean Lacau St-Guily and Professor Sophie Perié completed a successful autologous myoblast transplantation clinical trial for Oculo-pharyngeal muscular dystrophy. This common scientific career was made possible thanks to the precious and always benevolent support of François Gros.