Reactome | SLC25A1 exchanges CIT and MAL

SLC25A1 exchanges CIT and MAL

Stable Identifier

R-HSA-75849

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol, mitochondrial inner membrane, mitochondrial matrix

Synonyms

SLC25A1 exports citrate from mitochondrion in exchange for malate, Transport of Citrate from Mitochondrial Matrix to cytosol

ReviewStatus

5/5

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SLC25A1, in the inner mitochondrial membrane, mediates the exchange of mitochondrial citrate for cytosolic malate (Gnoni et al., 2009; reviewed in Zara et al., 2022). Mutations in SLC25A1 can cause neonatal-onset encephalopathy (D2L2AD, MIM:615182; Edvarson et al., 2013; Majd et al., 2018; reviewed by Zara et al., 2023) and a form of congenital myasthenic syndrome (CMS23, MIM:618197; Chaouch et al., 2014). SLC25A1 citrate export together with acetyl-CoA production by ACLY are sometimes referred to as the citrate-malate shuttle (for a review see Guo et al., 2023).

Literature References

PubMed ID	Title	Journal	Year
35842872	Multiple roles played by the mitochondrial citrate carrier in cellular metabolism and physiology Zara, V, Ferramosca, A, Assalve, G	Cell Mol Life Sci	2022
37051670	Determiners of cell fates: the tricarboxylic acid cycle versus the citrate-malate shuttle Meng, Y, Lu, Z, Luo, S, Guo, D, He, H	Protein Cell	2023
37209873	Insights into the malfunctioning of the mitochondrial citrate carrier: Implications for cell pathology Zara, V, Ferramosca, A, Assalve, G	Biochim Biophys Acta Mol Basis Dis	2023
29031613	Pathogenic mutations of the human mitochondrial citrate carrier SLC25A1 lead to impaired citrate export required for lipid, dolichol, ubiquinone and sterol synthesis King, MS, Kunji, ERS, Smith, AC, Majd, H	Biochim Biophys Acta Bioenerg	2018
23393310	Agenesis of corpus callosum and optic nerve hypoplasia due to mutations in SLC25A1 encoding the mitochondrial citrate transporter Elpeleg, O, Kellner, Y, Schechter, A, Palmieri, L, Shaag, A, Saada, A, Fraenkel, ND, Edvardson, S, Scarcia, P, Frumkin, A, Korman, SH, Segel, R, Pierri, CL, Soiferman, D, Pines, O, Jalas, C, Porcelli, V	J. Med. Genet.	2013
19787704	The mitochondrial citrate carrier: metabolic role and regulation of its activity and expression Siculella, L, Geelen, MJ, Priore, P, Gnoni, GV	IUBMB Life	2009
26870663	Mutations in the Mitochondrial Citrate Carrier SLC25A1 are Associated with Impaired Neuromuscular Transmission Cossins, J, Müller, JS, Palmieri, L, Abicht, A, Bushby, K, Chaouch, A, Evangelista, T, Beeson, D, Töpf, A, Edvardson, S, Horvath, R, Elpeleg, O, Porcelli, V, von der Hagen, M, Lochmüller, H, Griffin, H, Pierri, CL, Huebner, A, Cox, D, Laval, SH, De Grassi, A, Scarcia, P, Senderek, J, Palace, J, Straub, V	J Neuromuscul Dis	2014