Reactome | SLC25A26 exchanges cytosolic AdoMet for mitochondrial AdoHcy

SLC25A26 exchanges cytosolic AdoMet for mitochondrial AdoHcy

Stable Identifier

R-HSA-8855062

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol, mitochondrial inner membrane, mitochondrial matrix

ReviewStatus

5/5

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SLC25A26 exchanges cytosolic AdoMet for mitochondrial AdoHcy

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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 Kishita, Y, Pajak, A, Bolar, NA, Marobbio, CM, Maffezzini, C, Miniero, DV, Monné, M, Kohda, M, Stranneheim, H, Murayama, K, Naess, K, Lesko, N, Bruhn, H, Mourier, A, Wibom, R, Nennesmo, I, Jespers, A, Govaert, P, Ohtake, A, Van Laer, L, Loeys, BL, Freyer, C, Palmieri, F, Wredenberg, A, Okazaki, Y, Wedell, A	Am. J. Hum. Genet.	2015
14674884	Identification of the human mitochondrial S-adenosylmethionine transporter: bacterial expression, reconstitution, functional characterization and tissue distribution Agrimi, G, Di Noia, MA, Marobbio, CM, Fiermonte, G, Lasorsa, FM, Palmieri, F	Biochem. J.	2004