Clark Richard, Griffiths Gillian M
Sir William Dunn School of Pathology, South Parks Road, Oxford OX1 3RE, UK.
Curr Opin Immunol. 2003 Oct;15(5):516-21. doi: 10.1016/s0952-7915(03)00113-4.
Lytic granules harbour many of the dangerous apoptosis-inducing molecules of the immune system, including perforin, granzymes and Fas ligand. Safe transport, storage and release of these lytic components is vital. As a secretory lysosome, the lytic granule is able to accomplish these roles, as well as conferring the lysosomal functions of cytotoxic T lymphocytes and natural killer cells. Secretory lysosomes are common to many other haemopoietic cells and also melanocytes. Many of the proteins used in lysosomal secretion are found in both melanocytes and hemopoietic cells, and are dysfunctional in genetic diseases with defects in these proteins. The genetically heterogeneous Hermansky-Pudlak syndrome represents an excellent model for revealing proteins involved in secretory lysosome functioning. However, studies of this disease reveal differences between the various different types of secretory lysosomes, including lytic granules.
杀伤性颗粒含有许多免疫系统中危险的诱导凋亡分子,包括穿孔素、颗粒酶和Fas配体。安全运输、储存和释放这些杀伤性成分至关重要。作为一种分泌性溶酶体,杀伤性颗粒能够完成这些任务,同时赋予细胞毒性T淋巴细胞和自然杀伤细胞溶酶体功能。分泌性溶酶体在许多其他造血细胞以及黑素细胞中都很常见。溶酶体分泌中使用的许多蛋白质在黑素细胞和造血细胞中都有发现,并且在这些蛋白质存在缺陷的遗传疾病中功能失调。基因异质性的赫尔曼斯基-普德拉克综合征是揭示参与分泌性溶酶体功能的蛋白质的一个极佳模型。然而,对这种疾病的研究揭示了各种不同类型的分泌性溶酶体之间的差异,包括杀伤性颗粒。