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Protein

Phosphate carrier protein, mitochondrial

Gene

SLC25A3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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:proton symporter activity Source: ProtInc
  • protein-containing complex binding Source: MGI

GO - Biological processi

Keywordsi

Biological processSymport, Transport

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

EuPathDBiHostDB:ENSG00000075415.12
HGNCiHGNC:10989 SLC25A3
MIMi600370 gene
neXtProtiNX_Q00325

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
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

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 dbSNP:rs104894375EnsemblClinVar.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

Protein-protein interaction databases

BioGridi111269, 143 interactors
IntActiQ00325, 45 interactors
MINTiQ00325
STRINGi9606.ENSP00000228318

Structurei

3D structure databases

ProteinModelPortaliQ00325
SMRiQ00325
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

Keywords - Domaini

Repeat, Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

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

Family and domain databases

Gene3Di1.50.40.10, 2 hits
InterProiView protein in InterPro
IPR018108 Mitochondrial_sb/sol_carrier
IPR023395 Mt_carrier_dom_sf
PfamiView protein in Pfam
PF00153 Mito_carr, 3 hits
SUPFAMiSSF103506 SSF103506, 1 hit
PROSITEiView protein in PROSITE
PS50920 SOLCAR, 3 hits

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 dbSNP:rs104894375EnsemblClinVar.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

Similar proteinsi

Entry informationi

Entry nameiMPCP_HUMAN
AccessioniPrimary (citable) accession number: Q00325
Secondary accession number(s): B3KS34, Q7Z7N7, Q96A03
Entry historyiIntegrated into UniProtKB/Swiss-Prot: December 1, 1992
Last sequence update: February 1, 1995
Last modified: May 23, 2018
This is version 185 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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