de Saint Basile G, Fischer A
Institut National de la Santé et de la Recherche Médicale (INSERM) U429, Hôpital Necker, 149 rue de Sèvres, 75015, Paris, France.
Curr Opin Immunol. 2001 Oct;13(5):549-54. doi: 10.1016/s0952-7915(00)00257-0.
Several human inherited immune disorders lead to the same fatal lymphoproliferative syndrome, called the hemophagocytic syndrome. Through defective perforin expression or transport, these disorders highlight the determinant role of the secretory cytotoxic pathway in the regulation of the immune response and in lymphocyte homeostasis. In addition, new effectors of this secretory pathway have been identified.
几种人类遗传性免疫疾病会导致同一种致命的淋巴细胞增生综合征，即噬血细胞综合征。通过穿孔素表达或运输缺陷，这些疾病凸显了分泌性细胞毒性途径在免疫反应调节和淋巴细胞稳态中的决定性作用。此外，已鉴定出该分泌途径的新效应分子。
