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首页> 外文期刊>Genomics >Genes required for functional glycosylation of dystroglycan are conserved in zebrafish.
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Genes required for functional glycosylation of dystroglycan are conserved in zebrafish.

机译：dystroglycan的功能性糖基化所需的基因在斑马鱼中是保守的。

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摘要

Mutations in human genes encoding proteins involved in alpha-dystroglycan glycosylation result in dystroglycanopathies: severe congenital muscular dystrophy phenotypes often accompanied by CNS abnormalities and ocular defects. We have identified the zebrafish orthologues of the seven known genes in this pathway and examined their expression during embryonic development. Zebrafish Large, POMT1, POMT2, POMGnT1, Fukutin, and FKRP show in situ hybridization patterns similar to those of dystroglycan, with broad expression throughout early development. By 30 h postfertilization (hpf), transcripts of all these genes are most prominent in the CNS, eye, and muscle, tissues that are predominantly affected in the dystroglycanopathies. In contrast, Large2 expression is more restricted and by 30 hpf is confined to the lens, cerebellum, and pronephric duct. We show that the monoclonal antibody IIH6, which recognizes a glycoform of dystroglycan, also detects the zebrafish protein. Injection of morpholino oligonucleotides against zebrafish Large2 resulted in loss of IIH6 immunostaining. These data indicate that the dystroglycan glycosylation pathway is conserved in zebrafish and suggest this organism is likely to be a useful model system for functional studies.
机译：编码参与α-dystroglycan糖基化的蛋白质的人类基因中的突变会导致dystroglycanopathies：严重的先天性肌营养不良症表型常伴有CNS异常和眼缺陷。我们已经确定了这条途径中七个已知基因的斑马鱼直向同源物，并检查了它们在胚胎发育过程中的表达。大型斑马鱼，POMT1，POMT2，POMGnT1，Fukutin和FKRP显示出与dystroglycan相似的原位杂交模式，并在整个早期发育过程中广泛表达。到受精后30 h（hpf），所有这些基因的转录本在中枢神经系统，眼睛和肌肉等营养失调主要受累的组织中最为突出。相比之下，Large2的表达受到更多限制，并且以30 hpf的作用仅限于晶状体，小脑和前肾导管。我们表明，识别dystroglycan的糖型的单克隆抗体IIH6，也检测斑马鱼蛋白。注射针对斑马鱼Large2的吗啉代寡核苷酸导致IIH6免疫染色的丧失。这些数据表明，dystroglycan糖基化途径在斑马鱼中是保守的，并表明该生物体可能是功能研究的有用模型系统。

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