Reactome | SLC4A4 cotransports Na+ with 3HCO3-

SLC4A4 cotransports Na+ with 3HCO3-

Stable Identifier

R-HSA-8878664

Type

Reaction [transition]

Species

Homo sapiens

Compartment

plasma membrane, cytosol, extracellular region

ReviewStatus

5/5

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SLC4A family members 4, 5, 7, and 9 can each couple the transport of bicarbonate (HCO3-) with sodium ions (Na+). SLC4A4 (aka NBCE1) is an electrogenic sodium/bicarbonate cotransporter with a Na+:HCO3- stoichiometry of 1:3, although it can also be 1:2 (Burnham et al. 1997). SLC4A4 encodes a protein which is expressed in the kidney and pancreas, with lesser expression in many other tissues (Abuladze et al. 1998). Mutations in SLC4A4 cause permanent isolated proximal renal tubular acidosis (pRTA) (results in accumulation of acid in the body due to a failure of the kidneys to effectively acidify urine) with ocular abnormalities (Igarashi et al. 1999).

Adenosylhomocysteinase 2 (AHCYL2) can upregulate ion-transporting proteins such as the electrogenic sodium bicarbonate cotransporter 1 (SLC4A4, aka NBCE1) (Yamaguchi & Ishikawa 2014).

Literature References

PubMed ID	Title	Journal	Year
9651366	Molecular cloning, chromosomal localization, tissue distribution, and functional expression of the human pancreatic sodium bicarbonate cotransporter Newman, D, Boorer, K, Pushkin, A, Hwang, J, Abuladze, N, Kurtz, I, Lee, I	J Biol Chem	1998
10545938	Mutations in SLC4A4 cause permanent isolated proximal renal tubular acidosis with ocular abnormalities Tozawa, F, Cha, SH, Igarashi, T, Satoh, H, Kanai, Y, Seki, G, Shiobara, M, Shimadzu, M, Endou, H, Kunimi, M, Mori, T, Tsukamoto, K, Inatomi, J, Sekine, T	Nat Genet	1999
9235899	Cloning and functional expression of a human kidney Na+:HCO3- cotransporter Wang, Z, Soleimani, M, Shull, GE, Burnham, CE, Amlal, H	J Biol Chem	1997
24472682	AHCYL2 (long-IRBIT) as a potential regulator of the electrogenic Na(+)-HCO3(-) cotransporter NBCe1-B Yamaguchi, S, Ishikawa, T	FEBS Lett.	2014