生理学-离子通道.ppt

生理学——离子通道 参赛者：杨金成，武琦 From：医学1405 ? voltage gated ion channel chemically gated ion channel Functions of Na-K pump keep the balance of ion inside and outside cells the basis of Ap and Rp keep the size of cells provide energy of secondary active transport resting time: C K+：CNa+= 50~100：1 efflux of K+ ions Net flux of k+=0 Diffusion force =electrical attractive force depolarization stimulus Gna open Na+ internal flow CNa+Ck+(100:1) Fast Na+ internal flow Aps upslope inactivate GNa+,Gk+ open RP efflux of K+ 钾离子病：常染色体显性良性家族性新生儿（BFNC)、1-型发作性共济失调、癫痫、阵发性舞蹈手足徐动症伴发作性共济失调等。 钠离子病：高钾型周期性麻痹、正常血钾型周期性麻痹、先天性副肌强直、先天性肌无力等。 钙离子病：家族性偏瘫型偏头痛、低钾型周期性瘫痪、恶性高热、中央脊髓性肌病等。 氯离子病：先天性肌强直(Thomsen型)、隐性遗传全身性肌强直(Becker型)、囊性纤维化病、遗传性肾结石病、 3-型Bartter综合征等。 恶性高热 特征：体温急剧升高，可达45℃～46℃；骨骼肌僵直，心动过速，血压异常，呼吸急促，血钾增高。 发病机制：在全麻过程中接触挥发性吸入麻醉药（如氟烷、安氟醚、异氟醚等）和去极化肌松药（琥珀酰胆碱）后出现骨骼肌强直性收缩，产生大量能量，导致体温持续快速增高，在没有特异性治疗药物的情况下，一般的临床降温措施难以控制体温的增高，最终可导致患者死亡。 Excitation-contraction coupling * * * Ion channel Categorise A Mechanism of action (Na-K pump) B Ion channelopathy C mechanically gated ion channel Na﹢,k﹢,ca2﹢，cl﹣..... Piezo protein (associated with Tactile sensation) Extracelluar:N2-Ach receptor、 GBAB receptor 、Gly receptor、NMDAR etc. Intracellular:cAMP、cGMP、G protein gated channel etc. active transport ——the fundamental mode ATPase inside: release 3Na+,bind 2K+ P.ATPase ATP ADP Drop Pi outside: Bind 3Na+，release 2K+ Na-K pump=ATPase The definition of IIon channelopathy: The disease which is