Neurodevelopmental study of congenital aganglionosis.

为了研究先天性巨结肠病的发展，我们研究了神经成分和细胞增殖相关抗原在突变小鼠胚胎（ls/ls ;致死斑点）肠中的表达。通过交配纯合子获得胚胎肠道标本（胚胎第10 -18天）。使用N-CAM、L1分子和增殖细胞相关核抗原（PCNA）的特异性抗体对表达进行免疫组织化学检查。主要结果：神经成分的免疫反应细胞从食管到肛门直肠，显示出E10和E14之间的单向迁移波。E10时，NCAM样和L1样分子免疫反应性表现出与对照组相同的模式（ls/+，+/+）。然而，从E10.5到E13.5，与对照组相比，免疫反应性仍然存在于更口侧。在E10.5和E14之间的突变体的免疫反应细胞的迁移受到抑制。PCNA样免疫反应的间充质细胞迁移前的神经嵴细胞，但在突变胚胎的免疫反应表现出微薄的模式。在神经嵴细胞迁移的间充质组织中发现了缺乏染色的模式，而不是在迁移细胞本身。这种受损的PCNA表达可能反映了迁移的微环境缺陷，使得神经嵴细胞不能正常迁移。

To study the development of Hirschsprung's disease, we investigated the expression of neural components and cell proliferation-related antigens in guts from mutant murine embryos (ls/ls ; lethal spotted). Embryonal gut specimens (embryonal day E10-18) were obtained by mating homozygotes ls/ls). The expression was examined immunohistochemically using antibodies specific for N-CAM, the L1 molecule, and proliferative cell-related nuclear antigen (PCNA). Main Results : Cells immunoreactive for neural components proceeded from the esophagus to the anorectum showing a one-way migratory wave between E10 and E14. The NCAM-like and L1 molecule-like immunoreactivity exhibited the same pattern as in the controls (ls/+, +/+) on E10. However, from E10.5 to E13.5, the immunoreactivity remained in the more oral side compared to that in controls. The migration of immunoreactive cells was suppressed between E10.5 and E14 in the mutants. The PCNA-like immunoreactivity on mesenchymal cells preceded migrating neural crest cells, but the immunoreactivity exhibited a meager pattern in the mutant embryos. Deficient staining patterns were found in the mesenchymal tissue for neural crest cell migration rather than in the migrating cells themselves. This impaired PCNA expression may reflect a deficient microenvironment for migration such that neural crest cells cannot migrate properly.