Open Journal of Molecular and Integrative Physiology

Volume 1, Issue 2 (August 2011)

ISSN Print: 2162-2159   ISSN Online: 2162-2167

Google-based Impact Factor: 1  Citations  

Physiological and pathophysiological roles of the electrogenic Na+-HCO3 cotransporter NBCe1

HTML  XML Download Download as PDF (Size: 277KB)  PP. 9-16  
DOI: 10.4236/ojmip.2011.12002    4,419 Downloads   11,063 Views  Citations

Affiliation(s)

.

ABSTRACT

The electrogenic Na+-HCO3 cotransporter NBCe1 encoded by SLC4A4 gene plays essential roles in the regulation of intracellular/extracellular pH. Three NBCe1 variants are thought to mediate distinct physiological roles with different modes of transport stoichiometry. Homozygous inactivating mutations in NBCe1 cause the isolated proximal renal tubular acidosis (pRTA) invariably associated with ocular abnormalities. Functional analyses indicate that more than 50% reduction in NBCe1 activity may be required to induce severe acidemia. Some of the pRTA- related NBCe1 mutations, which show defective me-mbrane expression in mammalian cells, are also associated with migraine. Dysregulation of local pH in brain due to the loss of NBCe1 activity in astrocytes may underlie this association. Two types of NBCe1 deficient animals, NBCe1 knockout and W516X knockin mice, have been reported. Both of them show severe acidemia and early lethality unless they are treated with alkali. In isolated renal proximal tubules from W516X knockin mice, both NBCe1 activity and the rate of bicarbonate absorption are severely reduced, confirming the essential role of NBCe1 in bicarbonate absorption from this nephron segment. In this review, we summarize the recent data about physiological and pathophysiological roles of NBCe1 in health and diseases.

Share and Cite:

Seki, G. , Yamada, H. , Horita, S. , Suzuki, M. , Yamazaki, O. , Paesschen, W. , Yang, S. and Lin, S. (2011) Physiological and pathophysiological roles of the electrogenic Na+-HCO3 cotransporter NBCe1. Open Journal of Molecular and Integrative Physiology, 1, 9-16. doi: 10.4236/ojmip.2011.12002.

Cited by

[1] O bloqueio em longo prazo do receptor do peptídeo-1 semelhante ao glucagon eleva os níveis de pressão sistólica em ratos hipertensos e normotensos
Thesis, 2019
[2] Regulation des elektrogenen Natrium Bikarbonat Kotransporters NBCe1 und der pH-Homöostase im Kolon der Maus
2016
[3] Roles of Akt and SGK1 in the Regulation of Renal Tubular Transport
BioMed Research International, 2015
[4] Cotransporter through Angiotension II-dependent WNK4-SPAK Phosphorylation Pathway. Sci Rep. 2016 Jan 5; 6: 18360. 4. Lin HJ1, Wu CP, Peng CK …
2014
[5] The acid-base regulation by renal proximal tubule
Open Journal of Molecular and Integrative Physiology, 2013
[6] Functional roles of electrogenic sodium bicarbonate cotransporter NBCe1 in ocular tissues
The open ophthalmology journal, 2012
[7] Book Chapters
2012

Copyright © 2025 by authors and Scientific Research Publishing Inc.

Creative Commons License

This work and the related PDF file are licensed under a Creative Commons Attribution 4.0 International License.