Chasis J A, Coulombel L, Conboy J, McGee S, Andrews K, Kan Y W, Mohandas N
Cell and Molecular Biology Division, Lawrence Berkeley Laboratory University of California, Berkeley 94720.
J Clin Invest. 1993 Jan;91(1):329-38. doi: 10.1172/JCI116189.
Erythroid differentiation is accompanied by dramatic alterations in morphology and membrane mechanical properties resulting, in large part, from reorganization of the membrane skeletal protein network. The 80-kD protein 4.1 is an important organizational component of this membrane skeleton. Recently, it has been recognized that multiple structural isoforms of 4.1 are encoded by a single gene via alternative pre-mRNA splicing, and that an upstream ATG can be spliced in and used for translation of high molecular weight 4.1. We are exploring the hypothesis that differentiation-associated switches in protein 4.1 structure play an important role in membrane reorganization. To study changes in 4.1 gene expression during normal human differentiation, we analyzed 4.1 protein and mRNA structure at various developmental stages. Using immunofluorescence microscopy, we observed high molecular weight 4.1 isoforms in preproerythroblasts producing punctate, predominantly cytoplasmic staining with a perinuclear area of intense fluorescence, while mature red cells expressed very little high molecular weight 4.1. Isoforms containing an alternatively expressed 102-nucleotide exon near the COOH terminus were abundant in both preproerythroblasts and mature cells but produced a punctate distribution of fluorescence over the entire preproerythroblast and intense membrane-associated fluorescence in the erythrocyte. Characterization of RNA by polymerase chain reaction and nuclease protection assays revealed a differentiation-associated switch in pre-mRNA splicing in the spectrin-actin binding domain. Since this domain plays a critical role in regulating membrane material properties, we speculate that this switch may be crucial to reorganization of the skeletal network during erythropoiesis. We conclude that 4.1 isoforms are differentially expressed and differentially localized during erythropoiesis, and that this isoform family is likely to have diverse functions during terminal differentiation.
红细胞分化伴随着形态和膜力学性质的显著改变,这在很大程度上是由膜骨架蛋白网络的重组导致的。80-kD蛋白4.1是这种膜骨架的重要组织成分。最近,人们认识到4.1的多种结构异构体是由单个基因通过可变前体mRNA剪接编码的,并且一个上游ATG可以被剪接入并用于高分子量4.1的翻译。我们正在探索这样一个假说,即蛋白4.1结构中与分化相关的转换在膜重组中起重要作用。为了研究正常人分化过程中4.1基因表达的变化,我们分析了不同发育阶段的4.1蛋白和mRNA结构。利用免疫荧光显微镜,我们在早幼红细胞前体细胞中观察到高分子量4.1异构体,产生点状的、主要是细胞质染色,核周区域有强烈荧光,而成熟红细胞中高分子量4.1表达很少。在COOH末端附近含有一个可变表达的102个核苷酸外显子的异构体在早幼红细胞前体细胞和成熟细胞中都很丰富,但在整个早幼红细胞前体细胞中产生点状荧光分布,在红细胞中产生强烈的膜相关荧光。通过聚合酶链反应和核酸酶保护试验对RNA进行表征,发现在血影蛋白-肌动蛋白结合域中前体mRNA剪接存在与分化相关的转换。由于该结构域在调节膜物质特性中起关键作用,我们推测这种转换可能对红细胞生成过程中骨架网络的重组至关重要。我们得出结论,4.1异构体在红细胞生成过程中差异表达且定位不同,并且这个异构体家族在终末分化过程中可能具有多种功能。