SLC13A3

Mammalian sodium-dicarboxylate cotransporters transport succinate and other Krebs cycle intermediates. They fall into 2 categories based on their substrate affinity: low affinity and high affinity. Both the low- and high-affinity transporters play an important role in the handling of citrate by the kidneys. The protein encoded by this gene represents the high-affinity form. Alternatively spliced transcript variants encoding different isoforms have been found for this gene, although the full-length nature of some of them have not been characterized yet. [provided by RefSeq]

Full Name

solute carrier family 13 (sodium-dependent dicarboxylate transporter), member 3

Function

High-affinity sodium-dicarboxylate cotransporter that accepts a range of substrates with 4-6 carbon atoms, such as the citric acid cycle intermediates succinate and alpha-ketoglutarate (2-oxoglutarate), as well as other compounds including N-acetyl-L-aspartate (PubMed:10794676, PubMed:10992006, PubMed:15561973, PubMed:17426067, PubMed:17356845, PubMed:24247155, PubMed:30635937).
Transports the dicarboxylate into the cell with a probable stoichiometry of 3 Na+ for 1 divalent dicarboxylate, rendering the process electrogenic (PubMed:10794676, PubMed:10992006).
Can transport citrate in a Na+-dependent manner, recognizing the divalent form of citrate rather than the trivalent form which is normally found in blood (PubMed:10794676).

Biological Process

Biological Process anion transmembrane transportManual Assertion Based On ExperimentIBA:GO_Central
Biological Process citrate transportISS:ARUK-UCL
Biological Process dicarboxylic acid transportManual Assertion Based On ExperimentIDA:ARUK-UCL
Biological Process glutathione transmembrane transportManual Assertion Based On ExperimentIDA:ARUK-UCL
Biological Process succinate transmembrane transportManual Assertion Based On ExperimentIDA:ARUK-UCL
Biological Process transport across blood-brain barrier1 PublicationNAS:ARUK-UCL

Cellular Location

Cell membrane

Involvement in disease

Leukoencephalopathy, acute reversible, with increased urinary alpha-ketoglutarate (ARLIAK):
An autosomal recessive disorder characterized by acute, reversible neurological deterioration during febrile illness. Patients exhibit reversible leukoencephalopathy and increased urinary excretion of alpha-ketoglutarate.

Topology

Cytoplasmic: 1-16
Helical: 17-37
Extracellular: 38-55
Helical: 56-76
Cytoplasmic: 77-82
Helical: 83-103
Extracellular: 104-137
Helical: 138-158
Cytoplasmic: 159-229
Helical: 230-250
Extracellular: 251-278
Helical: 279-299
Cytoplasmic: 300-336
Helical: 337-357
Extracellular: 358-372
Helical: 373-393
Cytoplasmic: 394-422
Helical: 423-443
Extracellular: 444-461
Helical: 462-482
Cytoplasmic: 483-505
Helical: 506-526
Extracellular: 527-546
Helical: 547-567
Cytoplasmic: 568-602