Reactome | SLC4A1,2,3 exchanges HCO3- for Cl-

SLC4A1,2,3 exchanges HCO3- for Cl-

Stable Identifier

R-HSA-425482

Type

Reaction [transition]

Species

Homo sapiens

Compartment

plasma membrane

Synonyms

Cl-/HCO3- exchanger transport

ReviewStatus

5/5

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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 Bruce, LJ, Anstee, DJ, Spring, FA, Tanner, MJ	J Biol Chem	1994
2594752	Cloning and characterization of band 3, the human erythrocyte anion-exchange protein (AE1) Lux, SE, John, KM, Kopito, RR, Lodish, HF	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 Kollert-Jöns, A, Wagner, S, Hübner, S, Appelhans, H, Drenckhahn, D	Am J Physiol	1993
9312167	Familial distal renal tubular acidosis is associated with mutations in the red cell anion exchanger (Band 3, AE1) gene Bruce, LJ, Cope, DL, Jones, GK, Schofield, AE, Burley, M, Povey, S, Unwin, RJ, Wrong, O, Tanner, MJ	J Clin Invest	1997
7923606	Molecular cloning, expression, and chromosomal localization of two isoforms of the AE3 anion exchanger from human heart Yannoukakos, D, Stuart-Tilley, A, Fernandez, HA, Fey, P, Duyk, G, Alper, SL	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 Jarolim, P, Rubin, HL, Brabec, V, Chrobak, L, Zolotarev, AS, Alper, SL, Brugnara, C, Wichterle, H, Palek, J	Blood	1995
3015590	Cloning and structural characterization of a human non-erythroid band 3-like protein Demuth, DR, Showe, LC, Ballantine, M, Palumbo, A, Fraser, PJ, Cioe, L, Rovera, G, Curtis, PJ	EMBO J	1986