Abnormal expression and processing of keratins in pupoid fetus (pf/pf) and repeated epilation (Er/Er) mutant mice.
Abnormal expression and processing of keratins in pupoid fetus (pf/pf) and repeated epilation (Er/Er) mutant mice.
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角质胎儿（PF/PF）和重复脱毛（ER/ER）突变小鼠中角质素的异常表达和加工。
DOI：
10.1083/jcb.105.4.1807
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发表时间：
1987-10
影响因子：
7.8
通讯作者：
Roop, D R
中科院分区：
文献类型：
--
作者：
Fisher, C;Jones, A;Roop, D R
小鼠的蛹状胎（pupoid fetus，pf）和重复脱毛（repeated epilepation，Er）突变导致纯合子表皮分化失败。表皮角蛋白的表达已遵循在PF/PF和Er/Er小鼠的二维凝胶电泳，免疫组化和Western印迹使用多克隆抗体，是单特异性的个别角蛋白多肽。我们的研究结果表明，分化特异性角蛋白（K1和K10）的表达延迟在PF/PF和Er/Er突变体，当这些角蛋白出现在以后的发展中，他们被定位在更深的层增厚的突变体表皮。相反，K6和K16，两种角蛋白，在正常表皮中发现的丰度低，是丰富的突变表皮。在新生突变体表皮中，发现K6和K16在最外层表皮细胞中最丰富，与K1和K10的分布相反。这些研究结果表明，这些增生角蛋白在突变小鼠中的表达可能发生在发育过程中和新生动物中的分化特异性角蛋白的排斥。分化，和角蛋白表达的一个明显的正常模式，发生时，整个pf/pf或Er/Er皮肤移植到正常小鼠。这些结果表明，PF和Er基因可能是系统性表达的，并且突变皮肤转移到“正常”环境中会导致正常表型的恢复。
The pupoid fetus (pf) and repeated epilation (Er) mutations of mice result in a failure of epidermal differentiation in homozygotes. Expression of the epidermal keratins has been followed in pf/pf and Er/Er mice by two-dimensional gel electrophoresis, and by immunohistochemistry and Western blotting using polyclonal antibodies that are monospecific for individual keratin polypeptides. Our results show that expression of the differentiation-specific keratins (K1 and K10) is delayed in both the pf/pf and Er/Er mutants and that, when these keratins do appear later in development, they are localized in the deeper layers of the thickened mutant epidermis. Conversely, K6 and K16, two keratins found in low abundance in normal epidermis, are abundant in mutant epidermis. In newborn mutant epidermis, K6 and K16 are found to be most abundant in the outermost epidermal cells, a distribution opposite to that of K1 and K10. These findings suggest that the expression of these hyperplastic keratins in mutant mice may occur to the exclusion of the differentiation-specific keratins both during development and in newborn animals. Differentiation, and an apparently normal pattern of keratin expression, occur when whole pf/pf or Er/Er skin is grafted to normal mice. These results suggest that the pf and Er genes may be expressed systemically and that transfer of the mutant skin to a "normal" environment results in the recovery of a normal phenotype.