Immunohistochemical studies of abnormal extrinsic nerve fibers in Hirschsprung's disease

HPC-1抗原最初被鉴定为中枢神经系统中的神经元特异性膜蛋白。因此，作为实验的第一步，免疫组织化学证实了HPC-1抗原在大鼠胃肠道神经系统中的存在。HPC-1被认为抑制多余的轴突侧枝发芽。利用hpc -1所属的胞吐相关蛋白抗体，免疫组化检测巨结肠病异常外源性神经纤维。6种胞吐相关蛋白均在正常乙状结肠中成功表达。另一方面，在结肠的神经节部分未检测到HPC-1，这可能解释了Hirschsprung病中结肠神经节部分多余的外神经纤维的原因。

The HPC-1 antigen was originally identified as a neuron-specific membrane protein in the central nervous system. So, as the first step of the experiments, the presence of HPC-1 antigen in the nervous system of the rat gastrointestinal tract was immunohistochemically confirmed. The HPC-1 is thought to suppress the excess axon-collateral sprouting.Abnormal extrinsic nerve fibers in Hirschsprung's disease was then immunohistochemically examined using antibody againt the exocytosis-related protein which the HPC-1belongs to. All six exocytosis-related Proteins were succesfully demonstrated in normal sigmoid colon. On the other hand, the HPC-1 was not detected in aganglionic portion of the colon, which may explain the reason of exess extrins nerve fibers in aganglionic portion of the colon in Hirschsprung's disease.