Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Tricarboxylate transport protein, mitochondrial

Gene

SLC25A1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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

Complete GO annotation...

Keywords - Biological processi

Transport

Enzyme and pathway databases

BioCyciZFISH:ENSG00000100075-MONOMER.
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

HGNCiHGNC:10979. SLC25A1.

Subcellular locationi

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

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • integral component of membrane Source: UniProtKB-KW
  • mitochondrial inner membrane Source: Reactome
  • nucleus Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Combined D-2- and L-2-hydroxyglutaric aciduria (D2L2AD)1 Publication
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 rs781925968dbSNPEnsembl.1
Natural variantiVAR_069494202M → T in D2L2AD. 1 PublicationCorresponds to variant rs782335811dbSNPEnsembl.1
Natural variantiVAR_069495282R → C in D2L2AD. 1 PublicationCorresponds to variant rs431905509dbSNPEnsembl.1
Natural variantiVAR_069496282R → G in D2L2AD. 1 PublicationCorresponds to variant rs431905509dbSNPEnsembl.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.
TopDownProteomicsiP53007.

PTM databases

iPTMnetiP53007.
PhosphoSitePlusiP53007.
SwissPalmiP53007.

Expressioni

Gene expression databases

BgeeiENSG00000100075.
CleanExiHS_SLC25A1.
ExpressionAtlasiP53007. baseline and differential.
GenevisibleiP53007. HS.

Organism-specific databases

HPAiHPA030183.

Interactioni

Protein-protein interaction databases

BioGridi112464. 46 interactors.
IntActiP53007. 25 interactors.
MINTiMINT-1153318.
STRINGi9606.ENSP00000215882.

Structurei

3D structure databases

ProteinModelPortaliP53007.
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

Contains 3 Solcar repeats.PROSITE-ProRule annotation

Keywords - Domaini

Repeat, Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0756. Eukaryota.
ENOG410YAYP. LUCA.
GeneTreeiENSGT00550000074856.
HOVERGENiHBG103009.
InParanoidiP53007.
KOiK15100.
OMAiKPKYRGF.
OrthoDBiEOG091G0KE7.
PhylomeDBiP53007.
TreeFamiTF105786.

Family and domain databases

Gene3Di1.50.40.10. 1 hit.
InterProiIPR002067. Mit_carrier.
IPR018108. Mitochondrial_sb/sol_carrier.
IPR023395. Mt_carrier_dom.
[Graphical view]
PfamiPF00153. Mito_carr. 3 hits.
[Graphical view]
PRINTSiPR00926. MITOCARRIER.
SUPFAMiSSF103506. SSF103506. 1 hit.
PROSITEiPS50920. SOLCAR. 3 hits.
[Graphical view]

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 rs781925968dbSNPEnsembl.1
Natural variantiVAR_069494202M → T in D2L2AD. 1 PublicationCorresponds to variant rs782335811dbSNPEnsembl.1
Natural variantiVAR_069495282R → C in D2L2AD. 1 PublicationCorresponds to variant rs431905509dbSNPEnsembl.1
Natural variantiVAR_069496282R → G in D2L2AD. 1 PublicationCorresponds to variant rs431905509dbSNPEnsembl.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.

Cross-referencesi

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.

3D structure databases

ProteinModelPortaliP53007.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi112464. 46 interactors.
IntActiP53007. 25 interactors.
MINTiMINT-1153318.
STRINGi9606.ENSP00000215882.

Protein family/group databases

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

PTM databases

iPTMnetiP53007.
PhosphoSitePlusiP53007.
SwissPalmiP53007.

Polymorphism and mutation databases

DMDMi20141931.

Proteomic databases

EPDiP53007.
MaxQBiP53007.
PaxDbiP53007.
PeptideAtlasiP53007.
PRIDEiP53007.
TopDownProteomicsiP53007.

Protocols and materials databases

DNASUi6576.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi6576.
DisGeNETi6576.
GeneCardsiSLC25A1.
H-InvDBHIX0040279.
HGNCiHGNC:10979. SLC25A1.
HPAiHPA030183.
MalaCardsiSLC25A1.
MIMi190315. gene.
615182. phenotype.
neXtProtiNX_P53007.
OpenTargetsiENSG00000100075.
Orphaneti356978. D,L-2-hydroxyglutaric aciduria.
98914. Presynaptic congenital myasthenic syndromes.
PharmGKBiPA35855.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0756. Eukaryota.
ENOG410YAYP. LUCA.
GeneTreeiENSGT00550000074856.
HOVERGENiHBG103009.
InParanoidiP53007.
KOiK15100.
OMAiKPKYRGF.
OrthoDBiEOG091G0KE7.
PhylomeDBiP53007.
TreeFamiTF105786.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000100075-MONOMER.
ReactomeiR-HSA-70263. Gluconeogenesis.
R-HSA-75105. Fatty Acyl-CoA Biosynthesis.

Miscellaneous databases

ChiTaRSiSLC25A1. human.
GeneWikiiSLC25A1.
GenomeRNAii6576.
PROiP53007.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000100075.
CleanExiHS_SLC25A1.
ExpressionAtlasiP53007. baseline and differential.
GenevisibleiP53007. HS.

Family and domain databases

Gene3Di1.50.40.10. 1 hit.
InterProiIPR002067. Mit_carrier.
IPR018108. Mitochondrial_sb/sol_carrier.
IPR023395. Mt_carrier_dom.
[Graphical view]
PfamiPF00153. Mito_carr. 3 hits.
[Graphical view]
PRINTSiPR00926. MITOCARRIER.
SUPFAMiSSF103506. SSF103506. 1 hit.
PROSITEiPS50920. SOLCAR. 3 hits.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiTXTP_HUMAN
AccessioniPrimary (citable) accession number: P53007
Secondary accession number(s): A8K8E8, Q9BSK6
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: February 11, 2002
Last modified: November 30, 2016
This is version 161 of the entry and version 2 of the sequence. [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

Documents

  1. Human chromosome 22
    Human chromosome 22: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.