第五章细胞内膜系统和囊泡转运.ppt

3、痛风 ：嘌呤代谢紊乱导致高尿酸血症，使尿酸以结晶形式沉结于关节、关节周围，并被白细胞所吞噬，被吞噬的尿酸盐结晶与溶酶体膜之间形成氢键，改变溶酶体膜的稳定性，使溶酶体中水解酶和组胺等炎症物质释放，引起白细胞自溶坏死，引发所在沉积组织急性炎症。被释放的脲酸盐又继续在组织沉积。 各类贮积症：如台-萨氏（Tay-Sachs）病，溶酶体中缺少氨基已糖酯酶A，不能将神经节甘脂GM2加工为GM3，导致神经节甘脂GM2积累，影响细胞功能，造成精神痴呆，2-6岁死亡。 台-萨氏综合症溶酶体的同心圆结构 四、过氧化物酶体与疾病 （一）原发性过氧化物酶体缺陷所致的遗传性疾病 遗传性无过氧化氢酶血症：抗感染能力下降，易发口腔炎等 Zellweger肝脑肾综合症：常染色体隐性遗传，肝肾细胞中过氧化物酶和过氧化氢酶缺乏，琥珀酸脱氢酶-黄素蛋白与CoQ之间的电子传递障碍。临床表现为严重肝功能障碍、重度骨骼肌张力减退、脑发育迟缓及癫痫等综合症状 （二）疾病过程中的过氧化物酶体的病理改变 甲状腺功能亢进、慢性酒精中毒和慢性低氧症：患者肝细胞中过氧化物酶体增多 甲状腺功能低下、高血脂症：过氧化物酶体数量少 * Figure 12-5. Hypotheses for the evolutionary origins of some membrane-bounded organelles. The origins of mitochondria, chloroplasts, ER, and the cell nucleus could explain the topological relationships of these intracellular compartments in eucaryotic cells. (A) A possible pathway for the evolution of the cell nucleus and the ER. In some bacteria the single DNA molecule is attached to an invagination of the plasma membrane, called a mesosome. Such an invagination in a very ancient procaryotic cell could have spread to form an envelope around the DNA while still allowing access of the DNA to the cell cytosol (as is required for DNA to direct protein synthesis). This envelope is presumed to have eventually pinched off completely from the plasma membrane, producing a nuclear compartment surrounded by a double membrane. As illustrated, the nuclear envelope is organized by a fibrous shell called the nuclear lamina and is penetrated by communicating channels called nuclear pore complexes. Because it is surrounded by two membranes that are in continuity where they are penetrated by these pores, the nuclear compartment is topologically equivalent to the cytosol. The lumen of the ER is continuous with the space between the inner and outer nuclear membranes and topologically equivalent to the extracellular space. (B) Mitochondria (and chloroplasts) are thought to have originated when a bacterium was engulfed by a larger pre-eucaryotic cell. They retain their autonomy. This may expla