第5章--物质的跨膜运输.pptVIP

Fig. Diagrammatic representation of neuromuscular transmission. (1) Action potential arriving at nerve terminal triggers opening of voltage gated calcium channels (VGCCs) and entry of calcium. (2) Rise in intracellular calcium triggers release of packets of acetylcholine (ACh). (3) Interaction of ACh with ACh receptors (AChR) depolarises post-synaptic membrane. (4) Voltage gated sodium channels (VGSCs) open, triggering muscle action potential. (5) ACh esterase (AChE) breaks ACh into acetyl and choline, which are taken up by the nerve terminal to be reformed into ACh. (6) Opening of voltage gated potassium channels (VGKCs) repolarises nerve terminal. * 细胞膜表面LDL受体基因突变，导致LDL受体缺失或异常，体内低密度脂蛋白代谢障碍，血浆总胆固醇和低密度脂蛋白-胆固醇（LDL-C）水平升高。 家族性高胆固醇血症(familial hypercholesterolemia)是一罕见的遗传性疾病，有家族性的特征，患者本身低密度脂蛋白(low density lipoprotein, LDL )胆固醇数值异常超高，若为同合子患者时其低密度脂蛋白(low density lipoprotein, LDL )胆固醇数值是正常人的4-6倍，通常LDL-C 为500-1200 mg/dL，甚至超过700 mg/dL，但三酸甘油酯(triglycerides)正常；也易于生命早期罹患心血管疾病。 因胆固醇沉淀而形成的黄色瘤(Xanthomas)经常会在生命早期中出现，局部性的分布于手肘(elbows)、 膝部(knees)以及手指间关节与阿基里氏腱(Achilles tendons)等位置。 　　该病的发病机制是细胞膜表面低密度脂蛋白受体 (LDLR)基因突变，导致LDLR缺如或异常，体内低密度 脂蛋白代谢障碍，血浆总胆固醇和低密度脂蛋白-胆固醇（LDL-C）水平升高。过量的LDL-C沉积于吞噬细胞和其它细胞，形成黄色瘤和粥样斑块，最终导致心血管疾病的发生。 * FIGURE 11-20a Membrane microdomains (rafts). (a) Stable associations of sphingolipids and cholesterol in the outer leaflet produce a microdomain, slightly thicker than other membrane regions, that is enriched with specific types of membrane proteins. GPI-linked proteins are common in the outer leaflet of these rafts, and proteins with one or several covalently attached long-chain acyl groups are common in the inner leaflet. Caveolin is especially common in inwardly curved rafts called caveolae (see Figure 11-21). Proteins with attached prenyl groups (such as Ras; see Box 12-2) tend to be excluded from rafts. FIGURE 11-21b Caveolin forces inward curvature of a membrane. Caveolae are small invaginations in the plasma membrane, as seen in (b) Each caveolin monomer has a