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Secretory-defect distal renal tubular acidosis is associated with transporter defect in H(+)-ATPase and anion exchanger-1

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Authors
Han, Jin Suk; Kim, Gheun-Ho; Kim, Jin; Jeon, Un Sil; Joo, Kwon Wook; Na, Ki Young; Ahn, Curie; Kim, Suhnggwon; Lee, Sang Eun; Lee, Jung Sang
Issue Date
2002-06-01
Publisher
American Society of Nephrology
Citation
J Am Soc Nephrol. 2002 Jun;13(6):1425-32.
Keywords
Acidosis, Renal Tubular/etiology/*metabolismAnion Exchange Protein 1, Erythrocyte/analysis/*physiologyBiopsyHumansHydrogen-Ion ConcentrationHypokalemia/metabolismImmunohistochemistryKidney/pathologyKidney Tubules, Distal/*metabolismProton-Translocating ATPases/analysis/*physiologySodium-Potassium-Exchanging ATPase/metabolism
Abstract
Recent progress in molecular physiology has permitted us to understand pathophysiology of various channelopathies at a molecular level. The secretion of H(+) from alpha-intercalated cells is mediated by apical plasma membrane H(+)-ATPase and basolateral plasma membrane anion exchanger-1 (AE1). Studies have demonstrated the lack of H(+)-ATPase immunostaining in the intercalated cells in a few patients with distal renal tubular acidosis (dRTA). Mutations in H(+)-ATPase and AE1 gene have recently been reported to cause dRTA. This study extends the investigation of the role of transporter defect in dRTA by using immunohistochemical methods. Eleven patients with hyperchloremic metabolic acidosis were diagnosed functionally to have secretory-defect dRTA: urine pH >5.5 during acidemia, normokalemia or hypokalemia, and urine-to-blood pCO(2) <25 mmHg during bicarbonaturia. Renal biopsy tissue was obtained from each patient, and immunohistochemistry was carried out using antibodies to H(+)-ATPase and AE1. For comparison, renal tissues from the patients who had no evidences of distal acidification defect by functional studies were used: four with glomerulopathy or tubulointerstitial nephritis (disease controls) and three from nephrectomized kidneys for renal cell carcinoma (normal controls). The H(+)-ATPase immunoreactivity in alpha-intercalated cells was almost absent in all of the 11 patients with secretory-defect dRTA. In addition, 7 of 11 patients with secretory-defect dRTA were accompanied by negative AE1 immunoreactivity. In both disease controls and normal controls, the immunoreactivity of H(+)-ATPase and AE1 was strong in alpha-intercalated cells. In conclusion, significant defect in acid-base transporters is the major cause of secretory-defect dRTA.
ISSN
1046-6673 (Print)
Language
English
URI
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=12039970

http://hdl.handle.net/10371/11611
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College of Medicine/School of Medicine (의과대학/대학원)Internal Medicine (내과학전공)Journal Papers (저널논문_내과학전공)
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