生物化学教学课件：Protein function.pptVIP

肉色的变化 §4.1 Oxygen-Binding Proteins Structure of Myoglobin and Hemoglobin O2-binding of Mb and Hb Factors effect on the O2-binding of Hb Hb Mutation disease Distribution of mutations in human hemoglobin Glu→Val Sickle cell anemia Sickle cell anemia is caused by a single amino acid replacement on the b subunit: Glu6→Val Glu→Val Q1: How Val differ to Glu? Q2: What may happen to the protein? Glu→Val Q1: How Val differ to Glu? Q2: What may happen to the protein? Electron micrograph of deoxy-Hb S fibers spilling out of a ruptured erythrocyte. The sickled cells are fragile. Their breakdown leads to an anemia that leaves the victim susceptible to infections and diseases. The elongated cells tend to block capillaries, causing inflammation and considerable pain By Linus Pauling in 1949. Sciences 1903 1987 Normal Trait Patient (Normal) (defective) Hb AS 杂合子个体具有明显的抗疟疾（malaria）特征 Hb AS 基因型频率在非洲部分地区相当高 Plasmodium (疟原虫）在红细胞内的代谢导致pH下降，引起 Bohr effect，出现更多的S型脱氧血红蛋白（deoxy-HbS），聚集形成纤维沉淀, 导致红细胞镰刀化，被脾脏清除。既Hb AS 杂合子个体感染疟原虫的红细胞易于被脾脏清除。 §4 Protein Function §4.1 Oxygen-Binding Proteins §4.2 Muscle contraction §4.3 Conclusion Muscle structure Muscle structure 横纹肌纤维肉质网 横小管 侧面液囊 肌原纤维 Muscle structure 肌(原纤维)节,肌小节 Muscle contraction H band Muscle contraction Actin molecules Tropomyosin molecules Troponin molecules Cross bridges (head groups of myosin molecules) Actin Tropomyosin Troponin Myosin molecule head groups Split by papain Thin filament Thin filament Thick filament 原肌球蛋白 肌钙蛋白 肌动蛋白 肌球蛋白 Myosin (1) (Two heavy chain) (four) Mr = 540, 000 MrH = 220, 000 MrL = 20, 000 ~325 nm Myosin (2) Myosin (3)：S1 structure Myosin (3)：S1 structure G actin(subunit) ? F actin (polymer) G actin ? F actin (2) = Ca2+ or Mg2+ G actin ? F actin (3) Interaction between myosin and F-actin   Step 1 Step 2 The “power stroke” model of muscle contraction (ATP-consuming Motor) Two conformations of the cross-bridge were detected in insect muscle (1965) Step 3 Step 4 Step 1 Tropomyos