SLC4A4 cotransports Na+ with 3HCO3-

Stable Identifier
R-HSA-8878664
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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
10545938 Mutations in SLC4A4 cause permanent isolated proximal renal tubular acidosis with ocular abnormalities

Igarashi, T, Inatomi, J, Sekine, T, Cha, SH, Kanai, Y, Kunimi, M, Tsukamoto, K, Satoh, H, Shimadzu, M, Tozawa, F, Mori, T, Shiobara, M, Seki, G, Endou, H

Nat Genet 1999
9651366 Molecular cloning, chromosomal localization, tissue distribution, and functional expression of the human pancreatic sodium bicarbonate cotransporter

Abuladze, N, Lee, I, Newman, D, Hwang, J, Boorer, K, Pushkin, A, Kurtz, I

J Biol Chem 1998
9235899 Cloning and functional expression of a human kidney Na+:HCO3- cotransporter

Burnham, CE, Amlal, H, Wang, Z, Shull, GE, Soleimani, M

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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
sodium:bicarbonate symporter activity of SLC4A4 [plasma membrane]
Physical Entity
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