Yuan Qiuping, Chiquet Brett T, Devault Laura, Warman Matthew L, Nakamura Yukio, Swindell Eric C, Hecht Jacqueline T
Department of Pediatrics, University of Texas Medical School at Houston, Houston, Texas, USA.
Genesis. 2012 Dec;50(12):871-81. doi: 10.1002/dvg.22051. Epub 2012 Aug 21.
Nonsyndromic cleft lip and palate (NSCLP), a common birth defect, affects 4,000 newborns in the US each year. Previously, we described an association between CRISPLD2 and NSCLP and showed Crispld2 expression in the murine palate. These results suggested that a perturbation in CRISPLD2 activity affects craniofacial development. Here, we describe crispld2 expression and the phenotypic consequence of its loss of function in zebrafish. crispld2 was expressed at all stages of zebrafish morphogenesis examined and localized to the rostral end by 1-day postfertilization. Morpholino knockdown of crispld2 resulted in significant jaw and palatal abnormalities in a dose-dependent manner. Loss of crispld2 caused aberrant patterning of neural crest cells (NCC) suggesting that crispld2 is necessary for normal NCC formation. Altogether, we show that crispld2 plays a significant role in the development of the zebrafish craniofacies and alteration of normal protein levels disturbs palate and jaw formation. These data provide support for a role of CRISPLD2 in NSCLP.
非综合征性唇腭裂(NSCLP)是一种常见的出生缺陷,在美国每年影响4000名新生儿。此前,我们描述了CRISPLD2与NSCLP之间的关联,并在小鼠腭部显示了Crispld2的表达。这些结果表明,CRISPLD2活性的扰动会影响颅面发育。在这里,我们描述了crispld2在斑马鱼中的表达及其功能丧失的表型后果。在检查的斑马鱼形态发生的所有阶段都有crispld2表达,到受精后1天时定位于吻端。通过吗啉代敲低crispld2会以剂量依赖的方式导致明显的颌骨和腭部异常。crispld2的缺失导致神经嵴细胞(NCC)的模式异常,这表明crispld2对于正常的NCC形成是必需的。总之,我们表明crispld2在斑马鱼颅面发育中起重要作用,正常蛋白质水平的改变会干扰腭部和颌骨的形成。这些数据为CRISPLD2在NSCLP中的作用提供了支持。