SLC25A11 exchanges 2-OG and MAL

Stable Identifier
R-HSA-376851
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
SLC25A11 exchanges alpha-ketoglutarate (2-oxoglutarate) and malate across the inner mitochondrial membrane, (S)-malate(out) + 2-oxoglutarate(in) => (S)-malate(in) + 2-oxoglutarate(out)
ReviewStatus
5/5
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The SLC25A11 transport protein in the inner mitochondrial membrane mediates the reversible exchange of mitochondrial alpha-ketoglutarate (2OG, 2-oxoglutarate) and cytosolic malate (MAL) (Booty et al., 2015; Kabe et al. 2006). Kinetic details were also inferred from studies of rat mitochondria under conditions in which only the one transport protein appears to be active (Sluse et al. 1973). The dimeric state of the transport protein is inferred from studies of its bovine homologue (Bisaccia et al. 1996). Mutations in SLC25A11 can cause adult-onset development of paragangliomas (PGL6, MIM:618464; Buffet et al., 2018).
Literature References
PubMed ID Title Journal Year
29431636 Germline Mutations in the Mitochondrial 2-Oxoglutarate/Malate Carrier SLC25A11 Gene Confer a Predisposition to Metastatic Paragangliomas

Gimenez-Roqueplo, AP, Burnichon, N, Favier, J, Buffet, A, Padilla-Girola, M, Habarou, F, Letouzé, E, Morin, A, Bertherat, J, Ottolenghi, C, Lefebvre, H, Castro-Vega, LJ, Lussey-Lepoutre, C, Raingeard, I, Tchara, L, Guilhem, I, Tran, T, Haissaguerre, M, Amar, L

Cancer Res 2018
16920706 Porphyrin accumulation in mitochondria is mediated by 2-oxoglutarate carrier

Azuma, M, Watanabe, H, Ohmori, M, Hirao, S, Handa, H, Sinouchi, K, Okura, I, Kabe, Y, Tsuboi, Y

J Biol Chem 2006
8597574 The formation of a disulfide cross-link between the two subunits demonstrates the dimeric structure of the mitochondrial oxoglutarate carrier

Mazzeo, M, Bisaccia, F, Capobianco, L, Zara, V, Palmieri, F, Iacobazzi, V

Biochim Biophys Acta 1996
4687397 Mechanism of the exchanges catalysed by the oxoglutarate translocator of rat-heart mitochondria. Kinetics of the external-product inhibition

Sluse, FE, Goffart, G, Liébecq, C

Eur J Biochem 1973
25637873 The mitochondrial dicarboxylate and 2-oxoglutarate carriers do not transport glutathione

Booty, LM, James, AM, King, MS, Kunji, ER, Thangaratnarajah, C, Majd, H, Murphy, MP

FEBS Lett 2015
Participants
Participates
Catalyst Activity

oxoglutarate:malate antiporter activity of SLC25A11 homodimer [mitochondrial inner membrane]

Orthologous Events
Cross References
Rhea
RHEA
Authored
Reviewed
Created
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