SLC4A1,2,3 exchanges HCO3- for Cl-

Stable Identifier
Reaction [transition]
Homo sapiens
Cl-/HCO3- exchanger transport
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The proteins responsible for the exchange of Cl- with HCO3- are members of the SLC4 (1-3) and SLC26 (3, 4, 6, 7 and 9) transporter families. The SLC26 members are discussed under the section "Multifunctional anion exchangers".

SLC4A1 (Band 3, AE1, anion exchanger 1) was the first bicarbonate transporter gene to be cloned and sequenced (Lux et al. 1989). It is ubiquitous throughout vertebrates and in humans, is present on erythrocytes and the basolateral surfaces of kidney cells. The erythrocyte and kidney forms are different isoforms of the same protein (Kollert-Jons et al. 1993). Variations in erythroid AE1 determine the Diego blood group system (Bruce et al. 1994). A more serious consequence of mutated erythroid AE1 is Hereditary spherocytosis (a disorder leading to haemolytic anaemia) (Jarolim et al. 1995). Defects in the kidney form of AE1 cause distal (type1) renal tubular acidosis (an inability to acidify urine) (Bruce et al. 1997).

SLC4A2 (Non-erythroid band 3-like protein, AE2, anion exchanger 2) is widely expressed and is considered to be the 'housekeeping' isoform of the bicarbonate transporters (Demuth et al. 1986). SLC4A3 (Cardiac/brain band 3-like protein, AE3) is expressed in heart and brain (Yannoukakos et al. 1994).
Literature References
PubMed ID Title Journal Year
8206915 Band 3 Memphis variant II. Altered stilbene disulfonate binding and the Diego (Dia) blood group antigen are associated with the human erythrocyte band 3 mutation Pro854-->Leu

Tanner, MJ, Bruce, LJ, Spring, FA, Anstee, DJ

J Biol Chem 1994
2594752 Cloning and characterization of band 3, the human erythrocyte anion-exchange protein (AE1)

Lodish, HF, Lux, SE, John, KM, Kopito, RR

Proc Natl Acad Sci U S A 1989
7506871 Anion exchanger 1 in human kidney and oncocytoma differs from erythroid AE1 in its NH2 terminus

Appelhans, H, Kollert-Jöns, A, Drenckhahn, D, Hübner, S, Wagner, S

Am J Physiol 1993
9312167 Familial distal renal tubular acidosis is associated with mutations in the red cell anion exchanger (Band 3, AE1) gene

Tanner, MJ, Burley, M, Unwin, RJ, Bruce, LJ, Wrong, O, Jones, GK, Povey, S, Schofield, AE, Cope, DL

J Clin Invest 1997
7923606 Molecular cloning, expression, and chromosomal localization of two isoforms of the AE3 anion exchanger from human heart

Duyk, G, Alper, SL, Stuart-Tilley, A, Yannoukakos, D, Fey, P, Fernandez, HA

Circ Res 1994
7530501 Mutations of conserved arginines in the membrane domain of erythroid band 3 lead to a decrease in membrane-associated band 3 and to the phenotype of hereditary spherocytosis

Brugnara, C, Brabec, V, Alper, SL, Rubin, HL, Chrobak, L, Wichterle, H, Jarolim, P, Zolotarev, AS, Palek, J

Blood 1995
3015590 Cloning and structural characterization of a human non-erythroid band 3-like protein

Palumbo, A, Rovera, G, Fraser, PJ, Ballantine, M, Showe, LC, Curtis, PJ, Cioe, L, Demuth, DR

EMBO J 1986
Catalyst Activity

inorganic anion exchanger activity of SLC4A1,2,3 [plasma membrane]

Orthologous Events
Cross References
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