1302070909090307 080909070309 0105 3 + M1 M2 S1 S2 S3S4S5S6 S1S2S3S4S5 S6 S1S2S3S4S5S6 S7 0105 3 + 2-0007ｨｬ0208ｨｰ, 1-08ｨｹ0907 4-0007ｨｬ0208ｨｰ, 2-08ｨｹ090701ｨｰ 5 00ｨｹｨｪ07ｨｦ 15 00ｨｹｨｪ07ｨｦ ｨｮ00ｨ ｨｨ0405ｨ ｨｮ00ｨｦｨｺ05 7 010807-07ｨｦｨｺ070006ｨｪ02ｨｦ02ｨｰ Kir 1-7 Inwardly Rectifying Potassium Channels Kv 0105 3 + 6-0007ｨｬ0208ｨｰ 1-08ｨｹ0907 ﾂｨ ｨｬ040507ｨｰ ｨ 00ｨｴ00ｨｦｨｬｨｹ000400ｨ ｨｰ Voltage gated KCNQ (Kv7.1-5) Eag 0105 3 + 6-0007ｨｬ0208ｨｰ 1-08ｨｹ0907 ﾂｨ ｨｬ040507ｨｰ ｨ 00ｨｴ00ｨｦｨｬｨｹ000400ｨ ｨｰ Ca ++ - activated SKCa1 SKCa2 SKCa3 IKCa1* *IKCa1 : intermediate conductance Ca++-activated channel * HCOO- HCOO- HCOO- HCOO- 7-0007ｨｬ0208ｨｰ 1-08ｨｹ0907 ｨｬ0200050504ｨｰ ｨ 00ｨｴｨｬｨｦｨｬｨｹ00040007ｨｰ Ca ++ -gated Eag (Kv10.x) Erg (Kv11.x) Elk (Kv12.x) Slo1 Slo2.1* Slo2.2 Slo3 Maxi-K BK-ｨ Kv1.x KCNA Shaker Kv2.x KCNB Shab Kv3.x KCNC Shaw Kv4.x KCND Shal 32 00ｨｹｨｪ07ｨｦ ｨｮ00ｨ ｨｨ0405ｨ ｨｮ00ｨｦｨｺ05
13Na + Cl - Cl - Na + Cl - Na + H +, -ATPase H + V-ATPase 0909 ﾂｨｦｨｺｨｹ 00ｨｬ07ｨｬｨ ｨ 08ｨｴ 02ｨｮ0802ｨｦ09ｨ ｨｬ06ｨｪ0701 Ca ++ Mg ++ Na + 080205ｨｦｨｺｨｹ 00ｨｬ07ｨｬｨ ｨ 08ｨｴ 02ｨｮ0802ｨｦ09ｨ ｨｬ06ｨｪ0701 0701ｨｪ010200ｨｦｨｺｨｹ ｨｺｨ ｨｦ 020507ｨｦ060102ｨｰ ｨ ｨｨ0907ｨｦｨｮ00ｨｦｨｺｨｹ (principal) NHE1 0701ｨｪ010200ｨｦｨｺｨｹ ｨｺｨ ｨｦ 020507ｨｦ060102ｨｰ ｨ ｨｨ0907ｨｦｨｮ00ｨｦｨｺｨｹ (A-Intercalated) NKCC1 Cl - /HCO 3 - Exchanger Na + Na + Na + Ca ++ Mg ++?
1300ｨ ｨｮｨｦｨｺ06ｨｰ ｨｮ01ｨｪｨｨ07ｨｺ02ｨｰ 0701ｨｪ010200ｨｦｨｺｨｹ ｨｮｨｴ0504ｨｪ0509ｨｦ07 ENaC principal 0701ｨｪｨｨ07ｨｺ02ｨｰ ｨ 08ｨ ｨｦ000705ｨｮ02ｨｰ ｨ 080709070507 ｨｺｨ 05080701 0701ｨｪ010200ｨｦｨｺｨｹ ｨｮｨｴ0504ｨｪ0509ｨｦ07 principal Na+ Na+ ROMK BK ｨ,091 BK ｨ,091 Intercalated Intercalated BK ｨ,094 Na+ BK ｨ,094 Na+ 000507ｨｦ060102ｨｰ ｨ ｨｨ0907ｨｦｨｮ00ｨｦｨｺｨｹ 000507ｨｦ060102ｨｰ ｨ ｨｨ0907ｨｦｨｮ00ｨｦｨｺｨｹ ENaC principal Na+ principal Na+ BK ｨ Intercalated BK ｨ Intercalated BK ｨ,094 Na+ BK ｨ,094 Na+
13Flow-dependent potassium secretion Volume and calcium-dependent 090105ｨｹｨｰ 0mv Na + Cl - Na + Cl - -20mv Na + -40mv Na + Electroneutral Electroneutral Electrogenic Electrogenic BK channels 0909 ﾂｨｦｨｺｨｹ 00ｨｬ07ｨｬｨ ｨ 08ｨｴ 02ｨｮ0802ｨｦ09ｨ ｨｬ06ｨｪ0701 080205ｨｦｨｺｨｹ 00ｨｬ07ｨｬｨ ｨ 08ｨｴ 02ｨｮ0802ｨｦ09ｨ ｨｬ06ｨｪ0701 0701ｨｪ010200ｨｦｨｺｨｹ ｨｺｨ ｨｦ 020507ｨｦ060102ｨｰ ｨ ｨｨ0907ｨｦｨｮ00ｨｦｨｺｨｹ (principal) 02ｨｦ05ｨｬ02ｨｮ07ｨｰ ﾂ060907ｨｰ Na + Na + 0308ｨ ｨｪｨ 0909ｨｹ0204ｨｮ04 01ｨ + 09ｨｪ00ｨ 0505ｨ 0007 08 + /01ｨ + Na +
13 ｰChloride Shunt ｱ 090105ｨｹｨｰ Na + Cl - -20mv Na + Cl - -40mv Na + P 4 Electroneutral Electrogenic Electrogenic Tight Junction 080205ｨｦｨｺｨｹ 00ｨｬ07ｨｬｨ ｨ 08ｨｴ 02ｨｮ0802ｨｦ09ｨ ｨｬ06ｨｪ0701 0701ｨｪ010200ｨｦｨｺｨｹ ｨｺｨ ｨｦ 020507ｨｦ060102ｨｰ ｨ ｨｨ0907ｨｦｨｮ00ｨｦｨｺｨｹ (principal) 02ｨｦ05ｨｬ02ｨｮ07ｨｰ ﾂ060907ｨｰ Na + Cl - Na +
13Na + Nedd4-2 Ub ligase Proteosome 26S ALDO ROMK KS-WNK1 NaCl Acive NCC dimer WNK1 NaCl WNK3 WNK4 Lysosome Inacive NCC dimer Adaptin 3 Sortilin Trafficking NCC Monomer Cl - Claudins P4 Cl -
13 NaCl Acive NCC dimer NaCl ROMK WNK3 WNK1 WNK4 Normal Familial Hyperkalemic Hypertension 07 Pseudohypoaldosteronism 0005080701 2, 07 0705ｨｪ010907ｨｬ07 Gordon Hyperkalemia, Hyperchloremia, Metabolic acidosis Thizide sensitive Lysosome Inacive NCC dimer Adaptin 3 Sortilin Trafficking NCC Monomer ROMK 000200ｨ 0505050602ｨｦｨｰ NaCl Acive NCC dimer NaCl WNK3 X WNK4 ﾁｨ ｨｬ040507 0809ｨｹｨｮ05040304 Inacive NCC dimer Lysosome X Adaptin 3 Sortilin Trafficking NCC Monomer X 01ｨ 0009ｨｦ070201ｨ ｨｦｨｮｨｨ04ｨｮ08ｨ Cl - Claudins P4 Cl -
13 ｰ08ｨ ｨｪｨ 05070805ｨｨ02ｨｦ02ｨｰ ｱ 000701 01020109ｨｦｨｺ0705 0701ｨｮ0007ｨｬｨ 0007ｨｰ
13 ｰ08ｨ ｨｪｨ 05070805ｨｨ02ｨｦ02ｨｰ ｱ 0004ｨｰ 08ｨ 0901ｨｦ05ｨｰ
13 ｰ08ｨ ｨｪｨ 05070805ｨｨ02ｨｦ02ｨｰ ｱ 00ｨｴｨｪ 01020209ｨｴｨｪ
130701ｨｪ010907ｨｬ07 Gordon
0701ｨｪ010907ｨｬ07 Gordon
130102ｨｪ0200ｨｦｨｺ07 03000209070006ｨｪ02ｨｦｨ 000701 0701ｨｪ0109ｨｹｨｬ0701 Gordon 03ｨｦｨｺ070002ｨｪ0705ｨｰ 09080209ｨｺｨ 05ｨｦｨ ｨｦｨｬｨｦｨｺ07ｨｰ 0908060900ｨ ｨｮ04ｨｰ
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13Andersen-Tawil syndrome is a multisystem inherited disease exhibiting periodic paralysis, cardiac arrhythmias, dysmorphic features (voltage gated potassium channels-kncnj2 gene) mimicking catecholaminergic polymorphic ventricular tachycardia. Hypertelorism Retrognathia Micrognathia Clinodactyly Syndactyly
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13Karl Raimund Popper, (1902-1994)
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13High K+ intake Bｨｺ ｨ /091 ENaC Wid type Aldo 2 Na + Blood 3Na + Na +, -ATPase BK091-0803 Bｨｺ ｨ ENaC Aldo Na + 2 High K+ intake Blood 3Na + Na +, -ATPase Urine Conecting tubule epithelium Urine Chloride shunt Cl - Blood Conecting tubule epithelium
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13Maxi Calcium-Activated Potassium (BK) Channels Pore gating domain N voltage sensing domain N C Mg ++ Mg ++ Ca ++ Ca ++ C Mg ++ RCK1 RCK2 (calcium bowl) Regulatory domain of Conductance C Karen Castillo, et al.pnas,2015;112:4809ｨc4814
13THAL 5-10 mv Claudin 1 7 KCNQ Rate-limiting factor Kir 1-7 Chloride Chanel Ka Barttin* 1 7 1 7 1 7 Calcium Sensing Receptor 1 7 Chloride Chanel Kb *Barttin:Bartter Syndrome infatile with sensorinural deafness
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1303ｨｺ 0004ｨｰ 050707ｨｰ 0306ｨ 090006ｨｬ02ｨｪ04 09050604ｨｮ04 0004ｨｰ 03ｨｦｨｮｨｹ010701 09ｨｮ0902ｨｮ00080701 ｨｺｨ ｨｦ 090806ｨｺｨｺ09ｨｦｨｮ04ｨｰ 08ｨ 05080701 ｨｮ0007 0701ｨｪ010200ｨｦｨｺｨｹ ｨｺｨ ｨｦ 000507ｨｦ060102ｨｰ 09ｨｨ0907ｨｦｨｮ00ｨｦｨｺｨｹ 07ｨｴ0504ｨｪ0509ｨｦ07 Cl - Ca ++ Ca ++ TRPV4 / TRPP2 (polycystine2) Complex 0004 ﾂｨ ｨｪ070201ｨ 08ｨｮｨｨ040007ｨｦ 0108ｨ 010507ｨｦ LTCC(VOCC) 09080708ｨｹ05ｨｴｨｮ04 N C N 0105 2 C Cl - Ca ++ Ca ++ Calmodulin Ca ++ Calmodulin Calmodulin RCK2 (calcium bowl) RCK1 Regulatory domain of Conductance Ca ++ IP3 Receptor ATP Sarco/Endoplasmatic Reticulum Ca ++ ATPase (SECRA) 03ｨｪ01070805ｨ ｨｮｨｬｨ 00ｨｦｨｺｨｹ 0108ｨｺ000107 Ryanodyne Receptor (RyR)
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13070807ｨｪ010105ｨｦｨｺ07 ｨ 0900040908ｨ 030808ｨｮｨｨｨｦｨ 0200ｨｺ0202ｨ 05ｨｦｨｺ07 ｨ 0900040908ｨ 0106020109ｨ 09ｨｪ0600020904 0200ｨｺ0202ｨ 05ｨｦｨｺ07 ｨ 0900040908ｨ 0409ｨｹｨｮｨｨｨｦｨ ｨｺ0500ｨｴ 0200ｨｺ0202ｨ 05ｨｦｨｺ07 ｨ 0900040908ｨ 0001ｨｮｨｦ07050700ｨｦｨｺ05 00ｨ ｨｮｨｦｨｺ07 040907ｨｬ07ｨｺ04ｨｰ 030808ｨｮｨｨｨｦｨ ｨｺ0500ｨｴ 0200ｨｺ0202ｨ 05ｨｦｨｺ07 ｨ 0900040908ｨ 04ｨ 090200ｨｺ0202ｨ 050801ｨ 09ｨｪ0600020904 ｨｮ0807ｨｪ010105ｨｦｨｺ07 ｨ 0900040908ｨ 070807ｨｪ010105ｨｦｨｺ06ｨｰ 01020109ｨ 000002ｨｦｨ ｨｺ07 0208ｨ 0207 01020109ｨ 000002ｨｦｨ ｨｺ07 ｨｮ01ｨｬ080802ｨｮ04
13070807ｨｪ010105ｨｦｨｺ07 ｨ 0900040908ｨ 0405ｨ 00ｨｦ07ｨｺ07ｨｦ05ｨｦｨ ｨｺ07 080907ｨｬ04ｨｺｨｦｨｺ07 0208ｨｦ02. 0405ｨ 00ｨｦ07ｨｺ07ｨｦ05ｨｦｨ ｨｺ07 080907ｨｬ04ｨｺｨｦｨｺ07 0208ｨｦ02. 070807ｨｪ010105ｨｦｨｺ07 ｨ 0900040908ｨ 01020109ｨ 000002ｨｦｨ ｨｺ07 0208ｨ 0207 9 ｨｺｨ ｨｦ 10 ｨｺ09ｨ ｨｪｨｦｨ ｨｺ06ｨｪ ｨｪ060109ｨｴｨｪ 01020109ｨ 000002ｨｦｨ ｨｺ07 0208ｨ 0207
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