SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix

Stable Identifier
Reaction [transition]
Homo sapiens
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Mitochondrial glutamate carriers 1 and 2 (SLC25A22, GC1 and SLC25A18, GC2) belong to the mitochondrial carrier family of transport proteins which shuttle substrates, metabolites and cofactors through the mitochondrial membrane, connecting the cytosol to the mitochondrial matrix. Glutamate (Glu) can be co-transported with H+ via SLC25A18 and SLC25A22, located on the inner mitochondrial membrane (Fiermonte et al. 2002). Defects in SLC25A22 can cause early infantile epileptic encephalopathy 3 (EIEE3; MIM:609304), a severe neonatal epilepsy characterised by a very early onset, erratic and fragmentary myoclonus. With no treatment available, children with EIEE3 die within 1 or 2 years of birth or survive in a vegetative state (Molinari et al. 2005).

Literature References
PubMed ID Title Journal Year
15592994 Impaired mitochondrial glutamate transport in autosomal recessive neonatal myoclonic epilepsy

Munnich, A, AttiƩ-Bitach, T, Kadhom, N, Palmieri, L, Rio, M, Rustin, P, Colleaux, L, Raas-Rothschild, A, Fiermonte, G, Molinari, F, Vekemans, M, Ben-Neriah, Z, Palmieri, F, Encha-Razavi, F

Am. J. Hum. Genet. 2005
11897791 Identification of the mitochondrial glutamate transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms

Agrimi, G, Fiermonte, G, Walker, JE, Palmieri, L, Palmieri, F, Todisco, S

J. Biol. Chem. 2002
Catalyst Activity

amino acid:proton symporter activity of SLC25A18,A22 [mitochondrial inner membrane]

Orthologous Events
Cross References
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