Tricarboxylate transport protein, mitochondrial

Definition:

Mitochondrial electroneutral antiporter that exports citrate from the mitochondria into the cytosol in exchange for malate. Also able to mediate the exchange of citrate for isocitrate, phosphoenolpyruvate, cis-aconitate and to a lesser extend cis-aconitate, maleate and succinate. In the cytoplasm citrate is important in the regulation of glycolysis through a feedback mechanism and in the production of acetyl-CoA which is needed for the synthesis of fatty acids, sterols, prostaglandins, dolichol and coenzyme Q (CoQ). Required for proper neuromuscular junction formation (Probable).

References:

[1]. Ana Pop, et al. An overview of combined D-2- and L-2-hydroxyglutaric aciduria: functional analysis of CIC variants. J Inherit Metab Dis. 2018 Mar;41(2):169-180. [Content Brief]

[2]. Amina Chaouch, et al. Mutations in the Mitochondrial Citrate Carrier SLC25A1 are Associated with Impaired Neuromuscular Transmission. J Neuromuscul Dis. 2014;1(1):75-90. [Content Brief]

[3]. Homa Majd, et al. Pathogenic mutations of the human mitochondrial citrate carrier SLC25A1 lead to impaired citrate export required for lipid, dolichol, ubiquinone and sterol synthesis. Biochim Biophys Acta Bioenerg. 2018 Jan;1859(1):1-7. [Content Brief]

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