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Protein

Tricarboxylate transport protein, mitochondrial

Gene

SLC25A1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Involved in citrate-H+/malate exchange. Important for the bioenergetics of hepatic cells as it provides a carbon source for fatty acid and sterol biosyntheses, and NAD+ for the glycolytic pathway.

GO - Molecular functioni

GO - Biological processi

Keywordsi

Biological processTransport

Enzyme and pathway databases

ReactomeiR-HSA-70263 Gluconeogenesis
R-HSA-75105 Fatty acyl-CoA biosynthesis

Protein family/group databases

TCDBi2.A.29.7.2 the mitochondrial carrier (mc) family

Names & Taxonomyi

Protein namesi
Recommended name:
Tricarboxylate transport protein, mitochondrial
Alternative name(s):
Citrate transport protein
Short name:
CTP
Solute carrier family 25 member 1
Tricarboxylate carrier protein
Gene namesi
Name:SLC25A1
Synonyms:SLC20A3
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 22

Organism-specific databases

EuPathDBiHostDB:ENSG00000100075.9
HGNCiHGNC:10979 SLC25A1
MIMi190315 gene
neXtProtiNX_P53007

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei29 – 46Helical; Name=1Sequence analysisAdd BLAST18
Transmembranei86 – 105Helical; Name=2Sequence analysisAdd BLAST20
Transmembranei129 – 143Helical; Name=3Sequence analysisAdd BLAST15
Transmembranei183 – 202Helical; Name=4Sequence analysisAdd BLAST20
Transmembranei224 – 241Helical; Name=5Sequence analysisAdd BLAST18
Transmembranei278 – 297Helical; Name=6Sequence analysisAdd BLAST20

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Combined D-2- and L-2-hydroxyglutaric aciduria (D2L2AD)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal recessive neurometabolic disorder characterized by neonatal-onset encephalopathy with severe muscular weakness, intractable seizures, respiratory distress, and lack of psychomotor development resulting in early death. Brain imaging shows abnormalities including enlarged ventricles, delayed myelination, and germinal layer cysts.
See also OMIM:615182
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_06949045P → L in D2L2AD. 1 Publication1
Natural variantiVAR_069491144E → Q in D2L2AD. 1 Publication1
Natural variantiVAR_069492167G → R in D2L2AD. 1 Publication1
Natural variantiVAR_069493193S → W in D2L2AD. 1 PublicationCorresponds to variant dbSNP:rs781925968Ensembl.1
Natural variantiVAR_077511198R → H in D2L2AD. 1 Publication1
Natural variantiVAR_069494202M → T in D2L2AD. 1 PublicationCorresponds to variant dbSNP:rs782335811Ensembl.1
Natural variantiVAR_069495282R → C in D2L2AD. 1 PublicationCorresponds to variant dbSNP:rs431905509EnsemblClinVar.1
Natural variantiVAR_069496282R → G in D2L2AD. 1 PublicationCorresponds to variant dbSNP:rs431905509EnsemblClinVar.1
Natural variantiVAR_069497297Y → C in D2L2AD. 1 Publication1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi6576
MalaCardsiSLC25A1
MIMi615182 phenotype
OpenTargetsiENSG00000100075
Orphaneti356978 D,L-2-hydroxyglutaric aciduria
98914 Presynaptic congenital myasthenic syndromes
PharmGKBiPA35855

Polymorphism and mutation databases

DMDMi20141931

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 13MitochondrionBy similarityAdd BLAST13
ChainiPRO_000001926214 – 311Tricarboxylate transport protein, mitochondrialAdd BLAST298

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei156PhosphoserineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiP53007
MaxQBiP53007
PaxDbiP53007
PeptideAtlasiP53007
PRIDEiP53007
ProteomicsDBi56567
TopDownProteomicsiP53007

PTM databases

iPTMnetiP53007
PhosphoSitePlusiP53007
SwissPalmiP53007

Expressioni

Gene expression databases

BgeeiENSG00000100075
CleanExiHS_SLC25A1
ExpressionAtlasiP53007 baseline and differential
GenevisibleiP53007 HS

Interactioni

Protein-protein interaction databases

BioGridi112464, 49 interactors
IntActiP53007, 45 interactors
MINTiP53007
STRINGi9606.ENSP00000215882

Structurei

3D structure databases

ProteinModelPortaliP53007
SMRiP53007
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Repeati23 – 111Solcar 1Add BLAST89
Repeati122 – 208Solcar 2Add BLAST87
Repeati218 – 303Solcar 3Add BLAST86

Sequence similaritiesi

Keywords - Domaini

Repeat, Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0756 Eukaryota
ENOG410YAYP LUCA
GeneTreeiENSGT00550000074856
HOVERGENiHBG103009
InParanoidiP53007
KOiK15100
OMAiLTFWSGA
OrthoDBiEOG091G0KE7
PhylomeDBiP53007
TreeFamiTF105786

Family and domain databases

Gene3Di1.50.40.10, 1 hit
InterProiView protein in InterPro
IPR002067 Mit_carrier
IPR018108 Mitochondrial_sb/sol_carrier
IPR023395 Mt_carrier_dom_sf
PfamiView protein in Pfam
PF00153 Mito_carr, 3 hits
PRINTSiPR00926 MITOCARRIER
SUPFAMiSSF103506 SSF103506, 1 hit
PROSITEiView protein in PROSITE
PS50920 SOLCAR, 3 hits

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P53007-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MPAPRAPRAL AAAAPASGKA KLTHPGKAIL AGGLAGGIEI CITFPTEYVK
60 70 80 90 100
TQLQLDERSH PPRYRGIGDC VRQTVRSHGV LGLYRGLSSL LYGSIPKAAV
110 120 130 140 150
RFGMFEFLSN HMRDAQGRLD STRGLLCGLG AGVAEAVVVV CPMETIKVKF
160 170 180 190 200
IHDQTSPNPK YRGFFHGVRE IVREQGLKGT YQGLTATVLK QGSNQAIRFF
210 220 230 240 250
VMTSLRNWYR GDNPNKPMNP LITGVFGAIA GAASVFGNTP LDVIKTRMQG
260 270 280 290 300
LEAHKYRNTW DCGLQILKKE GLKAFYKGTV PRLGRVCLDV AIVFVIYDEV
310
VKLLNKVWKT D
Length:311
Mass (Da):34,013
Last modified:February 11, 2002 - v2
Checksum:iF1341629924953D6
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti26G → E in AAB08515 (PubMed:8666394).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_06949045P → L in D2L2AD. 1 Publication1
Natural variantiVAR_069491144E → Q in D2L2AD. 1 Publication1
Natural variantiVAR_069492167G → R in D2L2AD. 1 Publication1
Natural variantiVAR_069493193S → W in D2L2AD. 1 PublicationCorresponds to variant dbSNP:rs781925968Ensembl.1
Natural variantiVAR_077511198R → H in D2L2AD. 1 Publication1
Natural variantiVAR_069494202M → T in D2L2AD. 1 PublicationCorresponds to variant dbSNP:rs782335811Ensembl.1
Natural variantiVAR_069495282R → C in D2L2AD. 1 PublicationCorresponds to variant dbSNP:rs431905509EnsemblClinVar.1
Natural variantiVAR_069496282R → G in D2L2AD. 1 PublicationCorresponds to variant dbSNP:rs431905509EnsemblClinVar.1
Natural variantiVAR_069497297Y → C in D2L2AD. 1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U25147 mRNA Translation: AAB08515.1
L76134 Genomic DNA Translation: AAL40091.1
L75823 mRNA Translation: AAL40090.1
AK292313 mRNA Translation: BAF85002.1
BC004980 mRNA Translation: AAH04980.1
BC008061 mRNA Translation: AAH08061.1
CCDSiCCDS13758.1
PIRiG01789
RefSeqiNP_005975.1, NM_005984.4
UniGeneiHs.111024

Genome annotation databases

EnsembliENST00000215882; ENSP00000215882; ENSG00000100075
GeneIDi6576
KEGGihsa:6576
UCSCiuc002zoz.6 human

Similar proteinsi

Entry informationi

Entry nameiTXTP_HUMAN
AccessioniPrimary (citable) accession number: P53007
Secondary accession number(s): A8K8E8, Q9BSK6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: February 11, 2002
Last modified: June 20, 2018
This is version 174 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

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