KEGG   PATHWAY: hsa04966Help
Entry
hsa04966                    Pathway                                
Name
Collecting duct acid secretion - Homo sapiens (human)
Description
One of the important roles of the collecting duct segment of the kidney nephron is acid secretion. As daily food intake loads acid into the body, urinary acid excretion is essential, and urine pH can drop as low as 4.5. The alpha-intercalated cell of collecting duct is the main responsible for hydrogen secretion into the urine. The carbon dioxide, which is generated in the cells and enters from the blood, is changed to carbonic acid. This carbonic acid is divided into hydrogen ion and bicarbonate ion. Intracellular CA II catalyses the formation of these ions. The hydrogen ion is secreted into the lumen by the luminal H(+)-ATPase. The bicarbonate ion is transported to the blood side by the anion exchanger type 1. Hydrogen ion in the lumen is trapped by urinary buffers. These include ammonium and phosphate.
Class
Organismal Systems; Excretory system
BRITE hierarchy
Pathway map
Collecting duct acid secretion
hsa04966

All organismsOrtholog table
Disease
H00241  
Combined proximal and distal renal tubular acidosis (RTA type 3)
H00428  
Distal renal tubular acidosis (RTA type 1)
Drug
D00355  
Lansoprazole (JAN/USP/INN)
D00455  
Omeprazole (JP16/USP/INN)
D00724  
Sodium rabeprazole (JP16)
D01207  
Omeprazole sodium (USAN)
D01920  
Tenatoprazole (JAN/INN)
D01984  
Esomeprazole magnesium hydrate (JAN)
D02593  
Pantoprazole Sodium Hydrate (JAN)
D04056  
Esomeprazole sodium (USAN)
D05259  
Omeprazole magnesium (USAN)
D05261  
Omeprazole sodium injection (JAN)
D05353  
Pantoprazole (USAN/INN)
D05900  
Timoprazole (INN)
D05901  
Picoprazole (INN)
D05906  
Leminoprazole (INN)
D07917  
Esomeprazole (INN)
D08463  
Rabeprazole (INN)
D08903  
Dexlansoprazole (INN/USAN)
D09339  
Esomeprazole potassium (USAN)
D10120  
Esomeprazole strontium (USAN)
Organism
Homo sapiens (human) [GN:hsa]
Gene
523  
ATP6V1A; ATPase, H+ transporting, lysosomal 70kDa, V1 subunit A [KO:K02145] [EC:3.6.3.14]
525  
ATP6V1B1; ATPase, H+ transporting, lysosomal 56/58kDa, V1 subunit B1 [KO:K02147] [EC:3.6.3.14]
526  
ATP6V1B2; ATPase, H+ transporting, lysosomal 56/58kDa, V1 subunit B2 [KO:K02147] [EC:3.6.3.14]
9550  
ATP6V1G1; ATPase, H+ transporting, lysosomal 13kDa, V1 subunit G1 [KO:K02152] [EC:3.6.3.14]
127124  
ATP6V1G3; ATPase, H+ transporting, lysosomal 13kDa, V1 subunit G3 [KO:K02152] [EC:3.6.3.14]
534  
ATP6V1G2; ATPase, H+ transporting, lysosomal 13kDa, V1 subunit G2 [KO:K02152] [EC:3.6.3.14]
245973  
ATP6V1C2; ATPase, H+ transporting, lysosomal 42kDa, V1 subunit C2 [KO:K02148] [EC:3.6.3.14]
528  
ATP6V1C1; ATPase, H+ transporting, lysosomal 42kDa, V1 subunit C1 [KO:K02148] [EC:3.6.3.14]
51382  
ATP6V1D; ATPase, H+ transporting, lysosomal 34kDa, V1 subunit D [KO:K02149] [EC:3.6.3.14]
90423  
ATP6V1E2; ATPase, H+ transporting, lysosomal 31kDa, V1 subunit E2 [KO:K02150] [EC:3.6.3.14]
529  
ATP6V1E1; ATPase, H+ transporting, lysosomal 31kDa, V1 subunit E1 [KO:K02150] [EC:3.6.3.14]
9296  
ATP6V1F; ATPase, H+ transporting, lysosomal 14kDa, V1 subunit F [KO:K02151] [EC:3.6.3.14]
8992  
ATP6V0E1; ATPase, H+ transporting, lysosomal 9kDa, V0 subunit e1 [KO:K02153] [EC:3.6.3.14]
155066  
ATP6V0E2; ATPase, H+ transporting V0 subunit e2 [KO:K02153] [EC:3.6.3.14]
10312  
TCIRG1; T-cell, immune regulator 1, ATPase, H+ transporting, lysosomal V0 subunit A3 [KO:K02154] [EC:3.6.3.14]
23545  
ATP6V0A2; ATPase, H+ transporting, lysosomal V0 subunit a2 [KO:K02154] [EC:3.6.3.14]
50617  
ATP6V0A4; ATPase, H+ transporting, lysosomal V0 subunit a4 [KO:K02154] [EC:3.6.3.14]
535  
ATP6V0A1; ATPase, H+ transporting, lysosomal V0 subunit a1 [KO:K02154] [EC:3.6.3.14]
527  
ATP6V0C; ATPase, H+ transporting, lysosomal 16kDa, V0 subunit c [KO:K02155] [EC:3.6.3.14]
9114  
ATP6V0D1; ATPase, H+ transporting, lysosomal 38kDa, V0 subunit d1 [KO:K02146] [EC:3.6.3.14]
245972  
ATP6V0D2; ATPase, H+ transporting, lysosomal 38kDa, V0 subunit d2 [KO:K02146] [EC:3.6.3.14]
495  
ATP4A; ATPase, H+/K+ exchanging, alpha polypeptide [KO:K01542] [EC:3.6.3.10]
496  
ATP4B; ATPase, H+/K+ exchanging, beta polypeptide [KO:K01543] [EC:3.6.3.10]
6521  
SLC4A1; solute carrier family 4, anion exchanger, member 1 (erythrocyte membrane protein band 3, Diego blood group) [KO:K06573]
10723  
SLC12A7; solute carrier family 12 (potassium/chloride transporters), member 7 [KO:K13627]
1188  
CLCNKB; chloride channel, voltage-sensitive Kb [KO:K05018]
760  
CA2; carbonic anhydrase II [KO:K01672] [EC:4.2.1.1]
Compound
C00001  
H2O
C00011  
CO2
C00014  
NH3
C00080  
H+
C00238  
Potassium cation
C00288  
HCO3-
C00698  
Cl-
C01353  
Carbonic acid
Reference
  Authors
Wagner CA, Kovacikova J, Stehberger PA, Winter C, Benabbas C, Mohebbi N
  Title
Renal acid-base transport: old and new players.
  Journal
Nephron Physiol 103:p1-6 (2006)
Reference
  Authors
Wagner CA, Devuyst O, Bourgeois S, Mohebbi N
  Title
Regulated acid-base transport in the collecting duct.
  Journal
Pflugers Arch 458:137-56 (2009)
Reference
  Authors
Koeppen BM
  Title
The kidney and acid-base regulation.
  Journal
Adv Physiol Educ 33:275-81 (2009)
Reference
PMID:9550618
  Authors
Hayashi M
  Title
Physiology and pathophysiology of acid-base homeostasis in the kidney.
  Journal
Intern Med 37:221-5 (1998)
Reference
  Authors
Fry AC, Karet FE
  Title
Inherited renal acidoses.
  Journal
Physiology (Bethesda) 22:202-11 (2007)
Reference
  Authors
Pereira PC, Miranda DM, Oliveira EA, Silva AC
  Title
Molecular pathophysiology of renal tubular acidosis.
  Journal
Curr Genomics 10:51-9 (2009)
Reference
  Authors
Rodriguez-Soriano J
  Title
New insights into the pathogenesis of renal tubular acidosis--from functional to  molecular studies.
  Journal
Pediatr Nephrol 14:1121-36 (2000)
Reference
  Authors
Alper SL
  Title
Genetic diseases of acid-base transporters.
  Journal
Annu Rev Physiol 64:899-923 (2002)
KO pathway
 
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