Coulombe Pierre A
Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Departments of Biological Chemistry, Oncology, and Dermatology, School of Medicine, Johns Hopkins University, Baltimore, MD 21205Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University, Baltimore, MD 21205
Mol Biol Cell. 2016 Sep 15;27(18):2807-10. doi: 10.1091/mbc.E15-09-0625.
In 1991, a set of transgenic mouse studies took the fields of cell biology and dermatology by storm in providing the first credible evidence that keratin intermediate filaments play a unique and essential role in the structural and mechanical support in keratinocytes of the epidermis. Moreover, these studies intimated that mutations altering the primary structure and function of keratin filaments underlie genetic diseases typified by cellular fragility. This Retrospective on how these studies came to be is offered as a means to highlight the 25th anniversary of these discoveries.
1991年，一系列转基因小鼠研究在细胞生物学和皮肤病学领域掀起了一场风暴，首次提供了可靠证据，证明角蛋白中间丝在表皮角质形成细胞的结构和机械支撑中发挥着独特而重要的作用。此外，这些研究还暗示，改变角蛋白丝一级结构和功能的突变是导致以细胞脆弱为特征的遗传性疾病的基础。本文回顾了这些研究的来龙去脉，以此作为纪念这些发现25周年的一种方式。
