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 ｨｦｨｺｨｹ 00ｨｬ07ｨ

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2 M1 M2 S1 S2 S3S4S5S6 S1S2S3S4S5 S6 S1S2S3S4S5S6 S ｨｬ0208ｨｰ, 1-08ｨｹｨｬ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ｨｦｨｺｨｦｨｺ070006ｨｪ02ｨｦ02ｨｰ Kir 1-7 Inwardly Rectifying Potassium Channels Kv 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 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 ｨｬ0208ｨｰ 1-08ｨｹ0907 ｨｬｨｰｨ 00ｨｴｨｬｨｦｨｬｨｹｨｰ 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

3 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 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ｨｪｨｪ010200ｨｦｨｺｨｹｨｺｨｨｦｨｦ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 ++?

4 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ｨｰｨｨｺｨｨｪ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 ｨｦ060102ｨｰｨｨｨ0907ｨｦｨｮ00ｨｦｨｺｨｹｨｦ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+

5 13Flow-dependent potassium secretion Volume and calcium-dependent 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ｨｪｨｪ010200ｨｦｨｺｨｹｨｺｨｨｦｨｦ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 ｨｨｪｨ 0909ｨｹ0204ｨｮ04 01ｨ + 09ｨｪ00ｨ 0505ｨ /01ｨ + Na +

6 13 ｰChloride Shunt ｱｨｹｨｰ Na + Cl - -20mv Na + Cl - -40mv Na + P 4 Electroneutral Electrogenic Electrogenic Tight Junction 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ｨｪｨｪ010200ｨｦｨｺｨｹｨｺｨｨｦｨｦ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 +

7 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 -

8 13 NaCl Acive NCC dimer NaCl ROMK WNK3 WNK1 WNK4 Normal Familial Hyperkalemic Hypertension 07 Pseudohypoaldosteronism , ｨｪ010907ｨｬ07 Gordon Hyperkalemia, Hyperchloremia, Metabolic acidosis Thizide sensitive Lysosome Inacive NCC dimer Adaptin 3 Sortilin Trafficking NCC Monomer ROMK ｨｨｦｨｰ NaCl Acive NCC dimer NaCl WNK3 X WNK4 ﾁｨｨｬｨｹｨｮ 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 -

9 13 ｰ08ｨｨｪｨｨｨ02ｨｦ02ｨｰｱｨｦｨｺｨｮ0007ｨｬｨ 0007ｨｰ

10 13 ｰ08ｨｨｪｨｨｨ02ｨｦ02ｨｰｱ 0004ｨｰ 08ｨ 0901ｨｦ05ｨｰ

11 13 ｰ08ｨｨｪｨｨｨ02ｨｦ02ｨｰｱ 00ｨｴｨｪｨｴｨｪ

12 130701ｨｪ010907ｨｬ07 Gordon

13 0701ｨｪ010907ｨｬ07 Gordon

14 130102ｨｪ0200ｨｦｨｺｨｪ02ｨｦｨｨｪ0109ｨｹｨｬ0701 Gordon 03ｨｦｨｺ070002ｨｪ0705ｨｰｨｺｨ 05ｨｦｨｨｦｨｬｨｦｨｺ07ｨｰｨｨｮ04ｨｰ

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16 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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18 13Karl Raimund Popper, ( )

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21 13High K+ intake Bｨｺｨ /091 ENaC Wid type Aldo 2 Na + Blood 3Na + Na +, -ATPase BK 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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25 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

26 13THAL 5-10 mv Claudin 1 7 KCNQ Rate-limiting factor Kir 1-7 Chloride Chanel Ka Barttin* Calcium Sensing Receptor 1 7 Chloride Chanel Kb *Barttin:Bartter Syndrome infatile with sensorinural deafness

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29 1303ｨｺ 0004ｨｰｨｰ 0306ｨｨｬ02ｨｪｨｮｨｰ 03ｨｦｨｮｨｹｨｮ0902ｨｮｨｺｨｨｦｨｺｨｺ09ｨｦｨｮ04ｨｰ 08ｨｨｮｨｪ010200ｨｦｨｺｨｹｨｺｨｨｦｨｦ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ｨｨｦ LTCC(VOCC) ｨｹ05ｨｴｨｮ04 N C N 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ｨｪｨｨｮｨｬｨ 00ｨｦｨｺｨｹ 0108ｨｺ Ryanodyne Receptor (RyR)

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33 ｨｪ010105ｨｦｨｺ07 ｨｨｨｮｨｨｨｦｨ 0200ｨｺ0202ｨ 05ｨｦｨｺ07 ｨｨｨ 09ｨｪｨｺ0202ｨ 05ｨｦｨｺ07 ｨｨ 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 ｨｨ 0001ｨｮｨｦｨｦｨｺ05 00ｨｨｮｨｦｨｺｨｬ07ｨｺ04ｨｰｨｮｨｨｨｦｨｨｺ0500ｨｴ 0200ｨｺ0202ｨ 05ｨｦｨｺ07 ｨｨ 04ｨｨｺ0202ｨｨ 09ｨｪｨｮ0807ｨｪ010105ｨｦｨｺ07 ｨｨｨｪ010105ｨｦｨｺ06ｨｰｨｨｦｨｨｺｨｨｨｦｨｨｺ07 ｨｮ01ｨｬ080802ｨｮ04

34 ｨｪ010105ｨｦｨｺ07 ｨｨ 0405ｨ 00ｨｦ07ｨｺ07ｨｦ05ｨｦｨｨｺｨｬ04ｨｺｨｦｨｺｨｦｨ 00ｨｦ07ｨｺ07ｨｦ05ｨｦｨｨｺｨｬ04ｨｺｨｦｨｺｨｦｨｪ010105ｨｦｨｺ07 ｨｨｨｨｦｨｨｺｨｨｺｨｨｦ 10 ｨｺ09ｨｨｪｨｦｨｨｺ06ｨｪｨｪ060109ｨｴｨｪｨｨｦｨｨｺｨ 0207

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R03_LAVAMAT_54610_01_oS_5kg_sy_ZP.bk

R03_LAVAMAT_54610_01_oS_5kg_sy_ZP.bk 13LAVAMAT 54610 05ｨｰｨｨ08ｨ 05ｨｹｨｪｨ 07 ｨｬｨ 03ｨｨｨｪｨ -ｨ 09ｨｰ06ｨｬｨｨｰ 06ｨｪ09ｨｰ08ｨｮｨｺ02ｨｨ07 0706 ｨｮ09ｨｰｨｨｪ0609ｨｺ02 ｨｨ 05ｨｺ090705ｨｮｨｨｰｨ 02ｨｨｨｨ 130709ｨ 03ｨ 02ｨｬｨ 07 0706ｨｺｨｮ07ｨｨｰ0205ｨｹｨｪｨｨ02ｨ, ｨｮ09ｨ 03ｨ 02ｨｬ04ｨｦ

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 ｨｦｨｺｨｹ 00ｨｬ07ｨ

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 ｨｦｨｺｨｹ 00ｨｬ07ｨ