Bolaños-Jiménez F, Bordais A, Behra M, Strähle U, Sahel J, Rendón A
Laboratoire de Physiopathologie Cellulaire et Moléculaire de la Rétine, EMI 99-18 Université Louis Pasteur, Strasbourg, France.
Mech Dev. 2001 Apr;102(1-2):239-41. doi: 10.1016/s0925-4773(01)00310-0.
Dystrophin, the protein defective in Duchenne muscular dystrophy (DMD), plays a critical role in the formation and maintenance of the neuromuscular junction. In addition to dystrophin, activation of internal promoters of the DMD gene leads to the production of several short products. Among these, Dp71, which consists of the C-terminal domain of dystrophin, is the most abundant product of the gene in non-muscle tissues and brain. In this report, we compare the temporal and regional expression patterns of dystrophin and Dp71 at different stages of embryonic development and during retinal differentiation in zebrafish. The Dp71 transcripts are the earliest to be expressed at 9-10 h post-fertilization (hpf) in the axial mesoderm. As development proceeds, intense Dp71 staining is observed in the notochord, the developing brain, the marginal regions of the somites and the eye primordium. At the completion of retinal differentiation, Dp71 is expressed in the ganglion and inner nuclear layers. Transcripts encoding dystrophin have a slightly later onset of expression, 13-14 hpf, and remain restricted to the transverse myosepta through all the developmental stages examined. The complementary patterns of expression of dystrophin and Dp71 suggest that these two proteins exert different functions during embryonic development in zebrafish.
肌营养不良蛋白是杜兴氏肌营养不良症(DMD)中存在缺陷的蛋白质,在神经肌肉接头的形成和维持中起关键作用。除了肌营养不良蛋白外,DMD基因内部启动子的激活还会导致几种短产物的产生。其中,由肌营养不良蛋白的C末端结构域组成的Dp71是该基因在非肌肉组织和大脑中最丰富的产物。在本报告中,我们比较了斑马鱼胚胎发育不同阶段以及视网膜分化过程中肌营养不良蛋白和Dp71的时空表达模式。Dp71转录本最早在受精后9-10小时(hpf)在轴中胚层中表达。随着发育的进行,在脊索、发育中的大脑、体节边缘区域和眼原基中观察到强烈的Dp71染色。在视网膜分化完成时,Dp71在神经节和内核层中表达。编码肌营养不良蛋白的转录本表达开始时间稍晚,为13-14 hpf,并且在所有检查的发育阶段都局限于横向肌隔。肌营养不良蛋白和Dp71的互补表达模式表明,这两种蛋白质在斑马鱼胚胎发育过程中发挥不同的功能。