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Targeted deletion of Ruvbl1 results in severe defects of epidermal development and perinatal mortality
Molecular and Cellular Pediatrics ( IF 2.4 ) Pub Date : 2021-02-12 , DOI: 10.1186/s40348-021-00111-1
Claudia Dafinger , Thomas Benzing , Jörg Dötsch , Bernhard Schermer , Max C. Liebau

Epidermal development is a complex process of regulated cellular proliferation, differentiation, and tightly controlled cell death involving multiple cellular signaling networks. Here, we report a first description linking the AAA+ (ATPases associated with various cellular activities) superfamily protein Ruvbl1 to mammalian epidermal development. Keratinocyte-specific Ruvbl1 knockout mice (Ruvbl1fl/flK14:Cretg) show a severe phenotype including dramatic structural epidermal defects resulting in the loss of the functional skin barrier and perinatal death. Thus, Ruvbl1 is a newly identified essential player for the development of differentiated epidermis in mice.

中文翻译:

有针对性的删除Ruvbl1会导致表皮发育和围产期死亡的严重缺陷

表皮发育是涉及多个细胞信号网络的调节细胞增殖,分化和严格控制的细胞死亡的复杂过程。在这里,我们报告第一个描述,将AAA +(与各种细胞活动相关的ATPase)超家族蛋白Ruvbl1与哺乳动物表皮发育联系起来。角质形成细胞特异性Ruvbl1基因敲除小鼠(Ruvbl1fl / flK14:Cretg)显示出严重的表型,包括剧烈的结构性表皮缺陷,导致功能性皮肤屏障的丧失和围产期死亡。因此,Ruvbl1是新近鉴定出的小鼠分化表皮发育的重要参与者。
更新日期:2021-02-15
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