SLC25A18,22 import L-Glu, H+

Stable Identifier
R-HSA-8875623
Type
Reaction [transition]
Species
Homo sapiens
Compartment
cytosol, mitochondrial inner membrane, mitochondrial matrix
Synonyms
SLC25A18,A22 cotransport Glu, H+ from cytosol to mitochondrial matrix
ReviewStatus
5/5
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SLC25A18,22 import L-Glu, H+
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Mitochondrial glutamate carriers 1 and 2 (SLC25A22, GC1, and SLC25A18, GC2) belong to the mitochondrial carrier family of transport proteins that 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 characterized by 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; reviewed in Spoto et al., 2021).
Literature References
PubMed ID Title Journal Year
34679360 Neonatal Seizures: An Overview of Genetic Causes and Treatment Options

Spoto, G, Saia, MC, Amore, G, Gitto, E, Loddo, G, Mainieri, G, Nicotera, AG, Di Rosa, G

Brain Sci 2021
15592994 Impaired mitochondrial glutamate transport in autosomal recessive neonatal myoclonic epilepsy

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

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

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

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
SLC25A18,22 import L-Glu, H+ (Bos taurus)
SLC25A18,22 import L-Glu, H+ (Caenorhabditis elegans)
SLC25A18,22 import L-Glu, H+ (Canis familiaris)
SLC25A18,22 import L-Glu, H+ (Danio rerio)
SLC25A18,22 import L-Glu, H+ (Dictyostelium discoideum)
SLC25A18,22 import L-Glu, H+ (Drosophila melanogaster)
SLC25A18,22 import L-Glu, H+ (Gallus gallus)
SLC25A18,22 import L-Glu, H+ (Mus musculus)
SLC25A18,22 import L-Glu, H+ (Rattus norvegicus)
SLC25A18,22 import L-Glu, H+ (Saccharomyces cerevisiae)
SLC25A18,22 import L-Glu, H+ (Sus scrofa)
SLC25A18,22 import L-Glu, H+ (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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