The role of scube genes in hedgehog signal transduction

癌症通常是由于胚胎发育过程中控制组织形成的基因调控失误或突变造成的。在胎儿正常生长过程中，特定的基因组合形成信号转导通路，协调细胞对环境的反应。其中一种途径被称为Hedgehog信号转导途径，它已被证明在恶性和正常组织中协调细胞分裂和模式。编码这一途径成分的基因在最常见的人类癌症形式中发生突变。了解这一途径是如何被调节的，对于设计治疗这些癌症的发生和发展的策略至关重要。在这项拨款中概述的研究计划是研究我们在实验室中发现的这一途径的新组成部分，在一个易于研究的脊椎动物模型中，斑马鱼胚胎。我们计划研究这类被称为sccube蛋白的蛋白质如何控制通路的激活。我们希望这将导致对这一过程的更全面的理解，同时有助于理解我们正在研究的肌肉细胞内模式过程的最终结果的本质

Cancer often results form the miss-regulation and-or mutation of genes that control tissue formation in the developing embryo. Particular sets of genes combine to form a signal transduction pathway that coordinates the cell's response to its environment during the course of normal fetal growth. One such pathway is called the Hedgehog signal transduction pathway which has been shown to coordinated cell division and patterning within malignant and normal tissues. Genes encoding components of this pathway are mutated in the most common forms of human cancers. Understanding how this pathway is regulated is critical to designing strategies to treat the onset and progression of these cancers. The studies outlined in this grant plan to study a new component of this pathway that we have identified in our laboratory, in an easy to study vertebrate model, the zebrafish embryo. We plan to study how this class of proteins, termed scube proteins, acts to control activation of the pathway. We hope this will lead to a fuller understanding of this process, and at the same time help understand the nature of the end result of the patterning process within the muscle cells that we are studying