SLC26A3,6 exchange Cl- for HCO3-

Stable Identifier
Reaction [transition]
Homo sapiens
Group 2 - Cl-/HCO3- exchanger transport
Locations in the PathwayBrowser
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout
The proteins responsible for the exchange of chloride (Cl-) with bicarbonate (HCO3-) are members of the SLC4 (1-3) and SLC26 (3 and 6) transporter families. SLC4 members are discussed in the section "Bicarbonate transporters".

SLC26A3 (Chloride anion exchanger, Down-regulated in adenoma, DRA) is expressed in the mucosa of the colon and helps mediate electrolyte and fluid absorption (Schweinfest et al. 1993). Defects in SLC26A3 cause congenital chloride diarrhea 1 (DIAR1), a disease characterized by watery stools containing an excess of chloride (Hoeglund et al. 1996). SLC26A6 encodes a protein involved in transporting chloride, oxalate, sulfate and bicarbonate (Waldegger et al. 2001). It is ubiquitously expressed, the highest levels present in kidney and pancreas.
Literature References
PubMed ID Title Journal Year
7683425 Identification of a colon mucosa gene that is down-regulated in colon adenomas and adenocarcinomas

Papas, TS, Schweinfest, CW, Suster, S, Kondoh, N, Henderson, KW

Proc Natl Acad Sci U S A 1993
11247665 Cloning and characterization of SLC26A6, a novel member of the solute carrier 26 gene family

Ramirez, A, Waldegger, S, Ayadi, H, Lang, F, Smith, RJ, Kubisch, C, Moschen, I

Genomics 2001
11834742 Functional characterization of three novel tissue-specific anion exchangers SLC26A7, -A8, and -A9

Makela, S, Kestila, M, Markovich, D, Kere, J, Saarialho-Kere, U, Lehtonen, E, Kujala, M, Lohi, H

J Biol Chem 2002
8896562 Mutations of the Down-regulated in adenoma (DRA) gene cause congenital chloride diarrhoea

Tomaszewski, L, Holmberg, C, Socha, J, Höglund, P, Haila, S, Airola, K, Karjalainen-Lindsberg, ML, Kere, J, de la Chapelle, A, Saarialho-Kere, U

Nat Genet 1996
Catalyst Activity

inorganic anion exchanger activity of SLC26A3,6 [plasma membrane]

Orthologous Events
Cross References
Cite Us!