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

Phosphate carrier protein, mitochondrial

Gene

SLC25A3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Transport of phosphate groups from the cytosol to the mitochondrial matrix. Phosphate is cotransported with H+. May play a role regulation of the mitochondrial permeability transition pore (mPTP).

GO - Molecular functioni

  • phosphate ion carrier activity Source: ProtInc
  • protein complex binding Source: MGI
  • symporter activity Source: UniProtKB-KW

GO - Biological processi

  • generation of precursor metabolites and energy Source: ProtInc
  • transport Source: ProtInc
Complete GO annotation...

Keywords - Biological processi

Symport, Transport

Enzyme and pathway databases

BioCyciZFISH:ENSG00000075415-MONOMER.

Protein family/group databases

TCDBi2.A.29.4.2. the mitochondrial carrier (mc) family.

Names & Taxonomyi

Protein namesi
Recommended name:
Phosphate carrier protein, mitochondrial
Alternative name(s):
Phosphate transport protein
Short name:
PTP
Solute carrier family 25 member 3
Gene namesi
Name:SLC25A3
Synonyms:PHC
ORF Names:OK/SW-cl.48
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 12

Organism-specific databases

HGNCiHGNC:10989. SLC25A3.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini50 – 63Mitochondrial intermembraneSequence analysisAdd BLAST14
Transmembranei64 – 86Helical; Name=1Sequence analysisAdd BLAST23
Topological domaini87 – 121Mitochondrial matrixSequence analysisAdd BLAST35
Transmembranei122 – 141Helical; Name=2Sequence analysisAdd BLAST20
Topological domaini142 – 161Mitochondrial intermembraneSequence analysisAdd BLAST20
Transmembranei162 – 183Helical; Name=3Sequence analysisAdd BLAST22
Topological domaini184 – 218Mitochondrial matrixSequence analysisAdd BLAST35
Transmembranei219 – 238Helical; Name=4Sequence analysisAdd BLAST20
Topological domaini239 – 261Mitochondrial intermembraneSequence analysisAdd BLAST23
Transmembranei262 – 284Helical; Name=5Sequence analysisAdd BLAST23
Topological domaini285 – 314Mitochondrial matrixSequence analysisAdd BLAST30
Transmembranei315 – 333Helical; Name=6Sequence analysisAdd BLAST19
Topological domaini334 – 362Mitochondrial intermembraneSequence analysisAdd BLAST29

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • integral component of plasma membrane Source: ProtInc
  • membrane Source: UniProtKB
  • mitochondrial inner membrane Source: ProtInc
  • mitochondrion Source: HPA
  • myelin sheath Source: Ensembl
  • nucleolus Source: HPA
  • nucleus Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Mitochondrial phosphate carrier deficiency (MPCD)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionFatal disorder of oxidative phosphorylation. Patients have lactic acidosis, hypertrophic cardiomyopathy and muscular hypotonia and die within the first year of life.
See also OMIM:610773
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_03285072G → E in MPCD. 1 PublicationCorresponds to variant rs104894375dbSNPEnsembl.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi5250.
MalaCardsiSLC25A3.
MIMi610773. phenotype.
OpenTargetsiENSG00000075415.
Orphaneti91130. Cardiomyopathy - hypotonia - lactic acidosis.
PharmGKBiPA35865.

Polymorphism and mutation databases

BioMutaiSLC25A3.
DMDMi730052.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 49MitochondrionBy similarityAdd BLAST49
ChainiPRO_000001925650 – 362Phosphate carrier protein, mitochondrialAdd BLAST313

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei99N6-acetyllysineCombined sources1
Modified residuei112N6-methyllysineCombined sources1
Modified residuei196PhosphotyrosineCombined sources1
Modified residuei209N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation, Methylation, Phosphoprotein

Proteomic databases

EPDiQ00325.
MaxQBiQ00325.
PaxDbiQ00325.
PeptideAtlasiQ00325.
PRIDEiQ00325.
TopDownProteomicsiQ00325-1. [Q00325-1]
Q00325-2. [Q00325-2]

PTM databases

iPTMnetiQ00325.
PhosphoSitePlusiQ00325.
SwissPalmiQ00325.

Expressioni

Gene expression databases

BgeeiENSG00000075415.
CleanExiHS_SLC25A3.
ExpressionAtlasiQ00325. baseline and differential.
GenevisibleiQ00325. HS.

Organism-specific databases

HPAiHPA045709.

Interactioni

Subunit structurei

Interacts with PPIF; the interaction is impaired by CsA.By similarity

GO - Molecular functioni

  • protein complex binding Source: MGI

Protein-protein interaction databases

BioGridi111269. 132 interactors.
IntActiQ00325. 41 interactors.
MINTiMINT-1156379.
STRINGi9606.ENSP00000228318.

Structurei

3D structure databases

ProteinModelPortaliQ00325.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Repeati63 – 147Solcar 1Add BLAST85
Repeati160 – 244Solcar 2Add BLAST85
Repeati261 – 339Solcar 3Add BLAST79

Sequence similaritiesi

Contains 3 Solcar repeats.PROSITE-ProRule annotation

Keywords - Domaini

Repeat, Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0767. Eukaryota.
ENOG410XPST. LUCA.
GeneTreeiENSGT00390000008708.
HOGENOMiHOG000164438.
HOVERGENiHBG024440.
InParanoidiQ00325.
KOiK15102.
OMAiLKFYALC.
OrthoDBiEOG091G0FJF.
PhylomeDBiQ00325.
TreeFamiTF314119.

Family and domain databases

Gene3Di1.50.40.10. 1 hit.
InterProiIPR018108. Mitochondrial_sb/sol_carrier.
IPR023395. Mt_carrier_dom.
[Graphical view]
PfamiPF00153. Mito_carr. 3 hits.
[Graphical view]
SUPFAMiSSF103506. SSF103506. 1 hit.
PROSITEiPS50920. SOLCAR. 3 hits.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform A (identifier: Q00325-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MFSSVAHLAR ANPFNTPHLQ LVHDGLGDLR SSSPGPTGQP RRPRNLAAAA
60 70 80 90 100
VEEQYSCDYG SGRFFILCGL GGIISCGTTH TALVPLDLVK CRMQVDPQKY
110 120 130 140 150
KGIFNGFSVT LKEDGVRGLA KGWAPTFLGY SMQGLCKFGF YEVFKVLYSN
160 170 180 190 200
MLGEENTYLW RTSLYLAASA SAEFFADIAL APMEAAKVRI QTQPGYANTL
210 220 230 240 250
RDAAPKMYKE EGLKAFYKGV APLWMRQIPY TMMKFACFER TVEALYKFVV
260 270 280 290 300
PKPRSECSKP EQLVVTFVAG YIAGVFCAIV SHPADSVVSV LNKEKGSSAS
310 320 330 340 350
LVLKRLGFKG VWKGLFARII MIGTLTALQW FIYDSVKVYF RLPRPPPPEM
360
PESLKKKLGL TQ
Length:362
Mass (Da):40,095
Last modified:February 1, 1995 - v2
Checksum:i78714C85931B22D5
GO
Isoform B (identifier: Q00325-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     54-83: QYSCDYGSGRFFILCGLGGIISCGTTHTAL → YSCEFGSAKYYALCGFGGVLSCGLTHTAV

Show »
Length:361
Mass (Da):39,959
Checksum:i43AD380B15553DA6
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_03285072G → E in MPCD. 1 PublicationCorresponds to variant rs104894375dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_00326954 – 83QYSCD…THTAL → YSCEFGSAKYYALCGFGGVL SCGLTHTAV in isoform B. 3 PublicationsAdd BLAST30

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X77337 Genomic DNA. Translation: CAB56611.1.
X77337 Genomic DNA. Translation: CAB56612.1.
X60036 mRNA. Translation: CAA42641.1.
AB064666 mRNA. Translation: BAB93517.1.
AK092689 mRNA. Translation: BAG52596.1.
CH471054 Genomic DNA. Translation: EAW97595.1.
CH471054 Genomic DNA. Translation: EAW97597.1.
BC000998 mRNA. Translation: AAH00998.1.
BC001328 mRNA. Translation: AAH01328.1.
BC003504 mRNA. Translation: AAH03504.1.
BC004345 mRNA. Translation: AAH04345.1.
BC006455 mRNA. Translation: AAH06455.1.
BC011574 mRNA. Translation: AAH11574.1.
BC011641 mRNA. Translation: AAH11641.1.
BC014019 mRNA. Translation: AAH14019.1.
BC015379 mRNA. Translation: AAH15379.2.
BC051367 mRNA. Translation: AAH51367.2.
CCDSiCCDS9065.1. [Q00325-2]
CCDS9066.1. [Q00325-1]
PIRiA53737.
B53737.
RefSeqiNP_002626.1. NM_002635.3. [Q00325-2]
NP_005879.1. NM_005888.3. [Q00325-1]
NP_998776.1. NM_213611.2. [Q00325-2]
UniGeneiHs.290404.

Genome annotation databases

EnsembliENST00000188376; ENSP00000188376; ENSG00000075415. [Q00325-2]
ENST00000228318; ENSP00000228318; ENSG00000075415. [Q00325-1]
ENST00000401722; ENSP00000383898; ENSG00000075415. [Q00325-2]
ENST00000549338; ENSP00000447740; ENSG00000075415. [Q00325-2]
ENST00000551917; ENSP00000447310; ENSG00000075415. [Q00325-1]
ENST00000552981; ENSP00000448708; ENSG00000075415. [Q00325-2]
GeneIDi5250.
KEGGihsa:5250.
UCSCiuc001tfm.4. human. [Q00325-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X77337 Genomic DNA. Translation: CAB56611.1.
X77337 Genomic DNA. Translation: CAB56612.1.
X60036 mRNA. Translation: CAA42641.1.
AB064666 mRNA. Translation: BAB93517.1.
AK092689 mRNA. Translation: BAG52596.1.
CH471054 Genomic DNA. Translation: EAW97595.1.
CH471054 Genomic DNA. Translation: EAW97597.1.
BC000998 mRNA. Translation: AAH00998.1.
BC001328 mRNA. Translation: AAH01328.1.
BC003504 mRNA. Translation: AAH03504.1.
BC004345 mRNA. Translation: AAH04345.1.
BC006455 mRNA. Translation: AAH06455.1.
BC011574 mRNA. Translation: AAH11574.1.
BC011641 mRNA. Translation: AAH11641.1.
BC014019 mRNA. Translation: AAH14019.1.
BC015379 mRNA. Translation: AAH15379.2.
BC051367 mRNA. Translation: AAH51367.2.
CCDSiCCDS9065.1. [Q00325-2]
CCDS9066.1. [Q00325-1]
PIRiA53737.
B53737.
RefSeqiNP_002626.1. NM_002635.3. [Q00325-2]
NP_005879.1. NM_005888.3. [Q00325-1]
NP_998776.1. NM_213611.2. [Q00325-2]
UniGeneiHs.290404.

3D structure databases

ProteinModelPortaliQ00325.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111269. 132 interactors.
IntActiQ00325. 41 interactors.
MINTiMINT-1156379.
STRINGi9606.ENSP00000228318.

Protein family/group databases

TCDBi2.A.29.4.2. the mitochondrial carrier (mc) family.

PTM databases

iPTMnetiQ00325.
PhosphoSitePlusiQ00325.
SwissPalmiQ00325.

Polymorphism and mutation databases

BioMutaiSLC25A3.
DMDMi730052.

Proteomic databases

EPDiQ00325.
MaxQBiQ00325.
PaxDbiQ00325.
PeptideAtlasiQ00325.
PRIDEiQ00325.
TopDownProteomicsiQ00325-1. [Q00325-1]
Q00325-2. [Q00325-2]

Protocols and materials databases

DNASUi5250.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000188376; ENSP00000188376; ENSG00000075415. [Q00325-2]
ENST00000228318; ENSP00000228318; ENSG00000075415. [Q00325-1]
ENST00000401722; ENSP00000383898; ENSG00000075415. [Q00325-2]
ENST00000549338; ENSP00000447740; ENSG00000075415. [Q00325-2]
ENST00000551917; ENSP00000447310; ENSG00000075415. [Q00325-1]
ENST00000552981; ENSP00000448708; ENSG00000075415. [Q00325-2]
GeneIDi5250.
KEGGihsa:5250.
UCSCiuc001tfm.4. human. [Q00325-1]

Organism-specific databases

CTDi5250.
DisGeNETi5250.
GeneCardsiSLC25A3.
HGNCiHGNC:10989. SLC25A3.
HPAiHPA045709.
MalaCardsiSLC25A3.
MIMi600370. gene.
610773. phenotype.
neXtProtiNX_Q00325.
OpenTargetsiENSG00000075415.
Orphaneti91130. Cardiomyopathy - hypotonia - lactic acidosis.
PharmGKBiPA35865.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0767. Eukaryota.
ENOG410XPST. LUCA.
GeneTreeiENSGT00390000008708.
HOGENOMiHOG000164438.
HOVERGENiHBG024440.
InParanoidiQ00325.
KOiK15102.
OMAiLKFYALC.
OrthoDBiEOG091G0FJF.
PhylomeDBiQ00325.
TreeFamiTF314119.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000075415-MONOMER.

Miscellaneous databases

ChiTaRSiSLC25A3. human.
GeneWikiiSLC25A3.
GenomeRNAii5250.
PROiQ00325.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000075415.
CleanExiHS_SLC25A3.
ExpressionAtlasiQ00325. baseline and differential.
GenevisibleiQ00325. HS.

Family and domain databases

Gene3Di1.50.40.10. 1 hit.
InterProiIPR018108. Mitochondrial_sb/sol_carrier.
IPR023395. Mt_carrier_dom.
[Graphical view]
PfamiPF00153. Mito_carr. 3 hits.
[Graphical view]
SUPFAMiSSF103506. SSF103506. 1 hit.
PROSITEiPS50920. SOLCAR. 3 hits.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiMPCP_HUMAN
AccessioniPrimary (citable) accession number: Q00325
Secondary accession number(s): B3KS34, Q7Z7N7, Q96A03
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 1, 1992
Last sequence update: February 1, 1995
Last modified: November 30, 2016
This is version 171 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 12
    Human chromosome 12: 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.