Q9H936 · GHC1_HUMAN

  • Protein
    Mitochondrial glutamate carrier 1
  • Gene
    SLC25A22
  • Status
    UniProtKB reviewed (Swiss-Prot)
  • Amino acids
  • Protein existence
    Evidence at protein level
  • Annotation score
    5/5

Function

function

Mitochondrial glutamate/H+ symporter. Responsible for the transport of glutamate from the cytosol into the mitochondrial matrix with the concomitant import of a proton (PubMed:11897791).
Plays a role in the control of glucose-stimulated insulin secretion (By similarity).

Catalytic activity

Kinetics

KM SUBSTRATE pH TEMPERATURE[C] NOTES EVIDENCE
4.85 mMglutamate
Vmax pH TEMPERATURE[C] NOTES EVIDENCE
63.1 μmol/min/gwith glutamate as substrate

GO annotations

all annotationsall molecular functionvirus receptor activitydna bindingrna bindingcytoskeletal motor activitycatalytic activitygtpase activitystructural molecule activitytransporter activitycytoskeletal protein bindinglipid bindingcyclase activityantioxidant activityoxidoreductase activitytransferase activityhydrolase activitylyase activityisomerase activityligase activityprotein tag activitycargo receptor activityhistone bindingprotein folding chaperonetranslation regulator activitynutrient reservoir activityreceptor ligand activitymolecular transducer activitymolecular adaptor activitytoxin activitycell adhesion mediator activitymolecular function regulator activityvirus coreceptor activitycatalytic activity, acting on a proteincatalytic activity, acting on dnacatalytic activity, acting on rnamolecular carrier activitytranscription regulator activitygeneral transcription initiation factor activitymolecular sensor activitymolecular sequestering activityatp-dependent activityother molecular functionall biological processmitotic cell cyclecytokinesiscytoplasmic translationimmune system processmuscle system processcirculatory system processrenal system processrespiratory system processcarbohydrate metabolic processgeneration of precursor metabolites and energydna replicationdna repairdna recombinationchromatin organizationdna-templated transcriptionregulation of dna-templated transcriptiontrna metabolic processprotein foldingprotein glycosylationamino acid metabolic processmodified amino acid metabolic processlipid metabolic processvitamin metabolic processsulfur compound metabolic processintracellular protein transportnucleocytoplasmic transportautophagyinflammatory responsemitochondrion organizationcytoskeleton organizationmicrotubule-based movementperoxisome organizationlysosome organizationchromosome segregationcell adhesionestablishment or maintenance of cell polarityprogrammed cell deathphotosynthesismrna metabolic processsnrna metabolic processvesicle-mediated transportreproductive processdigestive system processsignalingcell differentiationprotein catabolic processextracellular matrix organizationregulatory ncrna-mediated gene silencingtelomere organizationcell junction organizationwound healingribosome biogenesiscilium organizationanatomical structure developmentcell motilitynervous system processendocrine processprotein maturationtransmembrane transportnucleobase-containing small molecule metabolic processhepaticobiliary system processmembrane organizationprotein-containing complex assemblycell wall organization or biogenesisnitrogen cycle metabolic processprotein localization to plasma membranedefense response to other organismdetoxificationmeiotic nuclear divisionmitotic nuclear divisionmitochondrial gene expressioncarbohydrate derivative metabolic processother biological processall cellular componentnuclear chromosomeextracellular regionextracellular spacecell wallnucleusnuclear envelopenucleoplasmchromosomenucleolusmitochondrionlysosomeendosomevacuoleperoxisomeendoplasmic reticulumgolgi apparatuslipid dropletmicrotubule organizing centercytosolribosomecytoskeletonplasma membraneciliumplastidthylakoidexternal encapsulating structureextracellular matrixcytoplasmic vesicleorganelleother cellular component
Cell color indicative of number of GO terms
AspectTerm
Cellular Componentmitochondrial inner membrane
Molecular Functionamino acid:proton symporter activity
Molecular FunctionL-aspartate transmembrane transporter activity
Molecular FunctionL-glutamate transmembrane transporter activity
Biological Processaspartate transmembrane transport
Biological ProcessL-glutamate transmembrane transport
Biological Processmalate-aspartate shuttle
Biological Processmonoatomic ion transport
Biological Processregulation of insulin secretion

Keywords

Enzyme and pathway databases

Protein family/group databases

Names & Taxonomy

Protein names

  • Recommended name
    Mitochondrial glutamate carrier 1
  • Short names
    GC-1
  • Alternative names
    • Glutamate/H(+) symporter 1
    • Solute carrier family 25 member 22

Gene names

    • Name
      SLC25A22
    • Synonyms
      GC1

Organism names

  • Taxonomic identifier
  • Taxonomic lineage
    Eukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Primates > Haplorrhini > Catarrhini > Hominidae > Homo

Accessions

  • Primary accession
    Q9H936
  • Secondary accessions
    • A8K366
    • C9J1H6
    • E9PJD3
    • E9PKB2
    • E9PL68

Proteomes

Organism-specific databases

Subcellular Location

Mitochondrion inner membrane
; Multi-pass membrane protein

Features

Showing features for transmembrane.

TypeIDPosition(s)Description
Transmembrane12-32Helical; Name=1
Transmembrane62-82Helical; Name=2
Transmembrane107-127Helical; Name=3
Transmembrane189-209Helical; Name=4
Transmembrane223-243Helical; Name=5
Transmembrane292-312Helical; Name=6

Keywords

Disease & Variants

Involvement in disease

Developmental and epileptic encephalopathy 3 (DEE3)

  • Note
    • The disease is caused by variants affecting the gene represented in this entry
  • Description
    A severe form of epilepsy characterized by frequent tonic seizures or spasms beginning in infancy with a specific EEG finding of suppression-burst patterns, characterized by high-voltage bursts alternating with almost flat suppression phases. DEE3 is characterized by a very early onset, erratic and fragmentary myoclonus, massive myoclonus, partial motor seizures and late tonic spasms. The prognosis is poor, with no effective treatment, and children with the condition either die within 1 to 2 years after birth or survive in a persistent vegetative state.
  • See also
    MIM:609304
Natural variants in DEE3
Variant IDPosition(s)ChangeDescription
VAR_022737206P>Lin DEE3; disrupts L-glutamate transporter activity; dbSNP:rs121918334

Features

Showing features for natural variant.

TypeIDPosition(s)Description
Natural variantVAR_022737206in DEE3; disrupts L-glutamate transporter activity; dbSNP:rs121918334

Variants

We now provide the "Disease & Variants" viewer in its own tab.

The viewer provides 466 variants from UniProt as well as other sources including ClinVar and dbSNP.

Go to variant viewer

Keywords

Organism-specific databases

Miscellaneous

Chemistry

Genetic variation databases

PTM/Processing

Features

Showing features for chain.

TypeIDPosition(s)Description
ChainPRO_00000906191-323Mitochondrial glutamate carrier 1

Proteomic databases

PTM databases

Expression

Tissue specificity

Expressed at high levels in brain, liver, and pancreas.

Gene expression databases

Organism-specific databases

Interaction

Protein-protein interaction databases

Miscellaneous

Structure

Family & Domains

Features

Showing features for repeat.

TypeIDPosition(s)Description
Repeat6-93Solcar 1
Repeat101-214Solcar 2
Repeat223-312Solcar 3

Sequence similarities

Keywords

Phylogenomic databases

Family and domain databases

Sequence

  • Sequence status
    Complete
  • Length
    323
  • Mass (Da)
    34,470
  • Last updated
    2001-03-01 v1
  • Checksum
    D0E06FD88E5A3198
MADKQISLPAKLINGGIAGLIGVTCVFPIDLAKTRLQNQQNGQRVYTSMSDCLIKTVRSEGYFGMYRGAAVNLTLVTPEKAIKLAANDFFRHQLSKDGQKLTLLKEMLAGCGAGTCQVIVTTPMEMLKIQLQDAGRIAAQRKILAAQGQLSAQGGAQPSVEAPAAPRPTATQLTRDLLRSRGIAGLYKGLGATLLRDVPFSVVYFPLFANLNQLGRPASEEKSPFYVSFLAGCVAGSAAAVAVNPCDVVKTRLQSLQRGVNEDTYSGILDCARKILRHEGPSAFLKGAYCRALVIAPLFGIAQVVYFLGIAESLLGLLQDPQA

Computationally mapped potential isoform sequences

There are 17 potential isoforms mapped to this entry

View all
EntryEntry nameGene nameLength
K4DIA2K4DIA2_HUMANSLC25A22114
K4DIA8K4DIA8_HUMANSLC25A22190
K4DIB0K4DIB0_HUMANSLC25A22103
K4DIB3K4DIB3_HUMANSLC25A22161
K4DIB4K4DIB4_HUMANSLC25A2265
K4DIB6K4DIB6_HUMANSLC25A22156
K4DIB8K4DIB8_HUMANSLC25A22177
A0A0D9SEI9A0A0D9SEI9_HUMANSLC25A22197
A0A0D9SG84A0A0D9SG84_HUMANSLC25A22117
A0A0D9SFA8A0A0D9SFA8_HUMANSLC25A2297
A0A0D9SFE1A0A0D9SFE1_HUMANSLC25A22247
E9PS95E9PS95_HUMANSLC25A22196
E9PQ36E9PQ36_HUMANSLC25A22134
A0A0A6YYN8A0A0A6YYN8_HUMANSLC25A2278
E9PJH7E9PJH7_HUMANSLC25A22313
E9PJY0E9PJY0_HUMANSLC25A2272
E9PI74E9PI74_HUMANSLC25A22127

Sequence caution

The sequence AAH24212.2 differs from that shown. Reason: Erroneous initiation

Keywords

Sequence databases

Nucleotide SequenceProtein SequenceMolecule TypeStatus
AJ428202
EMBL· GenBank· DDBJ
CAD21007.1
EMBL· GenBank· DDBJ
mRNA
AK023106
EMBL· GenBank· DDBJ
BAB14407.1
EMBL· GenBank· DDBJ
mRNA
AK290481
EMBL· GenBank· DDBJ
BAF83170.1
EMBL· GenBank· DDBJ
mRNA
AP006621
EMBL· GenBank· DDBJ
-Genomic DNA No translation available.
BC019033
EMBL· GenBank· DDBJ
AAH19033.1
EMBL· GenBank· DDBJ
mRNA
BC023545
EMBL· GenBank· DDBJ
AAH23545.1
EMBL· GenBank· DDBJ
mRNA
BC024212
EMBL· GenBank· DDBJ
AAH24212.2
EMBL· GenBank· DDBJ
mRNA Different initiation

Genome annotation databases

Similar Proteins

Disclaimer

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