家装电工基础知识.pptVIP

2 防御功能 L的防御功能主要体现在几种吞噬泡中.如,位于肝脾等血通道.(1)吞噬清除抗原抗体复合体(2) 吞噬细菌病毒等.(3)机体感染发炎部位巨噬细胞的吞噬作用. 死细胞的清除主要是由巨噬细胞完成. 巨噬细胞的L非常多,L的酶协同过氧化氢等强氧化剂共同作用杀死细菌.由于巨噬细胞较多的吞噬作用,所以也可以看到巨噬细胞中有较多的残余小体.这可能导致巨噬细胞的短寿. 3 L的其他生理功能. (1) 作为消化器官为细胞提供营养 (2) 在分泌腺细胞中,参与分泌过程的调节 如 分泌细胞吞噬储存在腺体内腔的甲状腺球蛋白,吞噬泡与L融合,甲状腺球蛋白被水解成甲状腺素,然后分泌到细胞外的 毛细血管中 (3) 两栖类发育蝌蚪尾巴的退化 (4) 受精作用 精 子 形 成 模 式 图 The acrosomal reaction during sea urchin fertilization Acrosomal profilacin The cortical reaction during sea urchin fertilization 三 L的发生 Gol中的磷酸转移酶是如何在ER向G转送众多的蛋白质中识别溶酶体酶蛋白?现在知道溶酶体酶蛋白存在识别信号研究表明这一信号不是一段肽链而是依赖于构象的信号区(signal patch)186。 Secretary lysosome 187 1 granzyme 2 不依赖M6P Lysosomes in Health and Disease If the cell does not produce a certain lysosomal enzyme or if an enzyme is not properly addressed in the Golgi apparatus, a lysosomal storage disease can result. These diseases are caused by a massive accumulation of material that should have been digested in the lysosome. The sorting of lysosomal enzymes in a normal cell (left). First, enzymes receive a chemical address label in the Golgi apparatus (a) and move in vesicles (b) to a lysosome (c). Any enzyme that is accidentally swept out of the cell (d) is recaptured and taken back to the lysosome (e). If some part of the process goes awry, a lysosomal storage disorder can result. For example, in Hurlers syndrome (center), an enzyme is not produced (f), and the lysosome therefore lacks that enzyme (g). If correctly tagged enzyme is added to the cells in test tubes (h), the cells can capture the enzyme and take it to the lysosome (i). In I-cell disease (right), enzymes are correctly made, but they are not tagged in the Golgi (j), and therefore are not sent to the lysosome. When such enzymes leave the cell (k), they cannot be recaptured (l) and so the lysosome remains empty (m). 小儿致死遗传性疾病---台-萨氏病 神经节苷脂------ GM2--------- 在神经细胞中GM2比较多 在正常情况下GM2的代谢如下: 水解神经节苷脂GM2需要?氨基己糖脂酶A 水解神经节苷脂GM2D的?氨基己糖脂酶A缺失 * * 第四节 溶酶体和过氧化物酶体 溶酶体（lysosome）的发现较晚，首先是由Christian de Duve (1955)