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

Stable Identifier
R-HSA-8875623
Type
Reaction [transition]
Species
Homo sapiens
Compartment
cytosol, mitochondrial inner membrane, mitochondrial matrix
ReviewStatus
5/5
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SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix
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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, Attié-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
Participants
Input
Output
Participates
as an event of
Catalyst Activity

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

Physical Entity
SLC25A18,A22 [mitochondrial inner membrane] (Homo sapiens)
Activity
amino acid:proton symporter activity (GO:0005280)
Orthologous Events
SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix (Bos taurus)
SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix (Caenorhabditis elegans)
SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix (Canis familiaris)
SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix (Danio rerio)
SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix (Dictyostelium discoideum)
SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix (Drosophila melanogaster)
SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix (Gallus gallus)
SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix (Mus musculus)
SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix (Rattus norvegicus)
SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix (Saccharomyces cerevisiae)
SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix (Sus scrofa)
SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix (Xenopus tropicalis)
Cross References
Rhea
Authored
Jassal, B  (2016-06-08)
Reviewed
D'Eustachio, P  (2016-07-15)
Created
Jassal, B  (2016-06-08)
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