Naslov
NBCe1: An electrogenic Na+ bicarbonate contransporter, in epithelia

Autori
Brady, Clayton T. ; Dugandžić, Aleksandra ; Paker, Mark D. ; Romero, Michael F.

Vrsta, podvrsta i kategorija rada
Poglavlja u knjigama, znanstveni

Knjiga
Studies of epithelial transporters and ion channels : Ion channels and transporters of epithelia in health and disease - Vol. 3, 2nd ed.

Urednik/ci
Hamilton, Kirk L. ; Devor, Daniel C.

Izdavač
Springer

Grad
New York (NY)

Godina
2021

Raspon stranica
93-123

ISBN
978-3-030-55454-5

ISSN
2625-252X

Ključne riječi
acid-base ; epithelia ; ion transport ; kidney ; pH regulation

Sažetak
The archetypal electrogenic Na+-bicarbonate (HCO3 −) cotransporter (NBCe1: SLC4A4) is one of five Na+-coupled bicarbonate transporters encoded by the SLC4 family of genes. NBCe1 cotransports 1 Na+ with 2 HCO3 − or, as an accumulation of evidence suggests, 1 Na+ and with a single carbonate (CO3 2−). NBCe1 action contributes to the buffering of blood plasma and intracellular pH (pHi) and also supports transepithelial HCO3 −/fluid secretion. The SLC4A4 gene expresses three major NBCe1 isoforms: NBCe1-A (expressed in renal proximal tubular epithelial cells), NBCe1-B (expressed in a variety of epithelial and excitable cells), and NBCe1-C (expressed in neurons and glial cells). Aside from distribution differences, each isoform has a unique combination of amino- and carboxy-terminal sequences which dictates differences in baseline Na+-2HCO3 − cotransport activity and regulation. In humans, recessive mutations in NBCe1 cause proximal renal tubular acidosis (pRTA) with ocular abnormalities: a devastating but rare disturbance of pH and fluid balance. This chapter will focus on the physiological and pathological roles of NBCe1-A and NBCe1-B in epithelial tissues.

Izvorni jezik
Engleski

Znanstvena područja
Temeljne medicinske znanosti

POVEZANOST RADA