Recombinant Human Tricarboxylate transport protein (SLC20A3), Truncated

Uniprot Id

P53007

Target Species

Human

Target Name

SLC25A1

Target Full Name

Tricarboxylate transport protein, mitochondrial

Target Function

Citrate transporter that mediates the exchange of mitochondrial citrate for cytosolic malate. Also able to mediate the exchange of citrate for isocitrate, phosphoenolpyruvate, cis- but not trans-aconitate and to a lesser extend maleate and succinate. Important for the bioenergetics of hepatic cells as it provides a carbon source for fatty acid and sterol biosyntheses, and NAD(+) for the glycolytic pathway. Required for proper neuromuscular junction formation.

Target Involvement

Combined D-2- and L-2-hydroxyglutaric aciduria (D2L2AD)

Target Subcellular Location

Mitochondrion inner membrane; Multi-pass membrane protein.

Target Protein Families

Mitochondrial carrier (TC 2.A.29) family

Target Research Area

Transport

Target Synonyms

Citrate transport protein; CTP; mitochondrial; SLC20A3; Slc25a1; solute carrier family 20 (mitochondrial citrate transporter), member 3; solute carrier family 25 (mitochondrial carrier, citrate transporter), member 1; Solute carrier family 25 member 1; Tricarboxylate carrier protein; Tricarboxylate transport protein; tricarboxylate transport protein, mitochondrial; TXTP_HUMAN

Target Background

This gene encodes a member of the mitochondrial carrier subfamily of solute carrier proteins. Members of this family include nuclear-encoded transporters that translocate small metabolites across the mitochondrial membrane. This protein regulates the movement of citrate across the inner membranes of the mitochondria. Mutations in this gene have been associated with combined D-2- and L-2-hydroxyglutaric aciduria. Pseudogenes of this gene have been identified on chromosomes 7, 11, 16, and 19. Alternative splicing results in multiple transcript variants.