Hedgehog(Hh)信号通路在动物发育过程中起重要作用。Hh信号通路的失调可导致发育的缺陷及肿瘤的发生。 Hh 通路活性的调节是多层次的,通路的转录因子Cubitus interruptus (Ci)是一个重要的调节靶点。我们发现了一新基因,命名为hrk,是维持Hh通路正常活性必需的，hrk RNAi导致Ci蛋白水平显著下调，由此导致Hh 通路活性下降,HRK调节Hh通路机制不清楚。 本项目以果蝇为模式生物,研究HRK 调节Hh通路的分子机制。该研究具有重要理论意义,可加深我们对Hh 通路调节的认识;同时由于Hh不正常下调可导致新生儿前脑无裂畸形（HPE），研究HRK 维持Hh通路的机制有助于揭示hrk 基因突变与HPE是否有关，如有关则该研究对临床工作中针对性治疗具有重大指导意义。

Hedgehog (Hh) signaling plays a vital role in animal development and diseases. Normal level Hh signaling is essential for animal embryonic development; Malregulation of the Hh signaling causes several types of human cancer. Hh signaling is controled by multilayer regulation, the transcription factor Ci of Hh pathway is one of the key controling sites.We found one novel gene, named hrk (Hh signaling regulation kinase) is necessary for Hh signaling mantainace.hrk RNAi downregulated Ci level and reduced Hh target gene ptc-lacZ expression.The underlying mechanism through which HRK regulates Hh signaling is elusive. We use Drosophila as a model to dissect how HRK modulates Hh signaling through manipulating Ci. For abnormal lower Hh signaling is related to Holoprosencephaly (HRE),our work may help to shed light on the cause of HRE.