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Sonic Hedgehog(Shh)基因属于Hedgehog(Hh)基因家族,该家族最早在果蝇体内被发现,进化上呈高度保守状态。Sonic Hedgehog定位在7号染色体长臂远端(7q36),其通过细胞表面特殊受体Patched(Ptc)和Smoothened(Smo)被接收和传导,从而激活锌指蛋白C i/G li家族。Sonic Hedgehog基因作为重要的形态发生素,在胚胎发育、机体器官组织形成的过程中发挥了重要的作用,它的缺失或者失活会导致一系列严重的遗传疾病。其与体节、神经管、消化道、头面部、上下肢芽的发育以及肿瘤形成等有密切关系。本文主要就Sonic Hedgehog基因及其在发育中的调控作用作一综述。
The Sonic Hedgehog (Shh) gene belongs to the Hedgehog (Hh) gene family, which was first found in Drosophila and was evolutionarily highly conserved. Sonic Hedgehog is located on the long arm distal to chromosome 7 (7q36) and is activated and activated by the zinc finger protein C i / G li family via the cell surface receptors Patched (Ptc) and Smoothened (Smo). Sonic Hedgehog gene, as an important morphogenetic hormone, plays an important role in embryonic development and organ tissue formation. Its absence or inactivation leads to a series of serious genetic diseases. The body section, neural tube, digestive tract, head and face, upper and lower limb bud development and tumor formation are closely related. This article mainly reviews the Sonic Hedgehog gene and its regulatory role in development.