SLC25A26 exchanges cytosolic AdoMet for mitochondrial AdoHcy

Stable Identifier
R-HSA-8855062
Type
Reaction
Species
Homo sapiens
Compartment
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SLC25A26 (S-adenosylmethionine mitochondrial carrier protein) associated with the inner mitochondrial membrane mediates the exchange of cytosolic AdoMet (S-adenosylmethionine) for mitochondrial AdoHcy (S-adenosylhomocysteine). The substrate specificity and countertransport function of SLC25A26 were established from studies of liposomes reconstituted with purified protein in vitro (Agrimi et al. 2004). SLC25A26 mutations that disrupt transport activity have been identified in patients with defects of mitochondrial function, consistent with a requirement for AdoMet uptake from the cytosol to support mitochondrial methylation reactions (Kishita et al. 2015).

Literature References
PubMed ID Title Journal Year
26522469 Intra-mitochondrial Methylation Deficiency Due to Mutations in SLC25A26

Wedell, A, Lesko, N, Okazaki, Y, Kishita, Y, Palmieri, F, Van Laer, L, Govaert, P, Nennesmo, I, Pajak, A, Monné, M, Marobbio, CM, Mourier, A, Ohtake, A, Kohda, M, Miniero, DV, Loeys, BL, Freyer, C, Naess, K, Maffezzini, C, Murayama, K, Jespers, A, Bruhn, H, Wredenberg, A, Wibom, R, Stranneheim, H, Bolar, NA

Am. J. Hum. Genet. 2015
14674884 Identification of the human mitochondrial S-adenosylmethionine transporter: bacterial expression, reconstitution, functional characterization and tissue distribution

Fiermonte, G, Agrimi, G, Lasorsa, FM, Palmieri, F, Marobbio, CM, Di Noia, MA

Biochem. J. 2004
Participants
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Catalyst Activity

S-adenosyl-L-methionine transmembrane transporter activity of SLC25A26 [mitochondrial inner membrane]

Orthologous Events
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