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Q00325

- MPCP_HUMAN

UniProt

Q00325 - MPCP_HUMAN

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Protein

Phosphate carrier protein, mitochondrial

Gene

SLC25A3

Organism
Homo sapiens (Human)
Status
Reviewed - 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

  1. phosphate ion carrier activity Source: ProtInc
  2. protein complex binding Source: MGI
  3. symporter activity Source: UniProtKB-KW

GO - Biological processi

  1. generation of precursor metabolites and energy Source: ProtInc
  2. phosphate ion transmembrane transport Source: GOC
  3. transport Source: ProtInc
Complete GO annotation...

Keywords - Biological processi

Symport, 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
ProteomesiUP000005640: Chromosome 12

Organism-specific databases

HGNCiHGNC:10989. SLC25A3.

Subcellular locationi

GO - Cellular componenti

  1. extracellular vesicular exosome Source: UniProt
  2. integral component of plasma membrane Source: ProtInc
  3. membrane Source: UniProtKB
  4. mitochondrial inner membrane Source: ProtInc
  5. mitochondrion Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Mitochondrial phosphate carrier deficiency (MPCD) [MIM:610773]: Fatal disorder of oxidative phosphorylation. Patients have lactic acidosis, hypertrophic cardiomyopathy and muscular hypotonia and die within the first year of life.1 Publication
Note: The disease is caused by mutations affecting the gene represented in this entry.
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti72 – 721G → E in MPCD. 1 Publication
VAR_032850

Keywords - Diseasei

Disease mutation

Organism-specific databases

MIMi610773. phenotype.
Orphaneti91130. Cardiomyopathy - hypotonia - lactic acidosis.
PharmGKBiPA35865.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 4949MitochondrionBy similarityAdd
BLAST
Chaini50 – 362313Phosphate carrier protein, mitochondrialPRO_0000019256Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei99 – 991N6-acetyllysine1 Publication
Modified residuei196 – 1961Phosphotyrosine1 Publication
Modified residuei209 – 2091N6-acetyllysineBy similarity

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

MaxQBiQ00325.
PaxDbiQ00325.
PRIDEiQ00325.

PTM databases

PhosphoSiteiQ00325.

Expressioni

Gene expression databases

BgeeiQ00325.
CleanExiHS_SLC25A3.
ExpressionAtlasiQ00325. baseline and differential.
GenevestigatoriQ00325.

Organism-specific databases

HPAiHPA045709.

Interactioni

Subunit structurei

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

Protein-protein interaction databases

BioGridi111269. 72 interactions.
IntActiQ00325. 30 interactions.
MINTiMINT-1156379.
STRINGi9606.ENSP00000228318.

Structurei

3D structure databases

ProteinModelPortaliQ00325.
SMRiQ00325. Positions 69-337.
ModBaseiSearch...
MobiDBiSearch...

Topological domain

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini50 – 6314Mitochondrial intermembraneSequence AnalysisAdd
BLAST
Topological domaini87 – 12135Mitochondrial matrixSequence AnalysisAdd
BLAST
Topological domaini142 – 16120Mitochondrial intermembraneSequence AnalysisAdd
BLAST
Topological domaini184 – 21835Mitochondrial matrixSequence AnalysisAdd
BLAST
Topological domaini239 – 26123Mitochondrial intermembraneSequence AnalysisAdd
BLAST
Topological domaini285 – 31430Mitochondrial matrixSequence AnalysisAdd
BLAST
Topological domaini334 – 36229Mitochondrial intermembraneSequence AnalysisAdd
BLAST

Transmembrane

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transmembranei64 – 8623Helical; Name=1Sequence AnalysisAdd
BLAST
Transmembranei122 – 14120Helical; Name=2Sequence AnalysisAdd
BLAST
Transmembranei162 – 18322Helical; Name=3Sequence AnalysisAdd
BLAST
Transmembranei219 – 23820Helical; Name=4Sequence AnalysisAdd
BLAST
Transmembranei262 – 28423Helical; Name=5Sequence AnalysisAdd
BLAST
Transmembranei315 – 33319Helical; Name=6Sequence AnalysisAdd
BLAST

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Repeati63 – 14785Solcar 1Add
BLAST
Repeati160 – 24485Solcar 2Add
BLAST
Repeati261 – 33979Solcar 3Add
BLAST

Sequence similaritiesi

Contains 3 Solcar repeats.PROSITE-ProRule annotation

Keywords - Domaini

Repeat, Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiNOG265500.
GeneTreeiENSGT00390000008708.
HOGENOMiHOG000164438.
HOVERGENiHBG024440.
InParanoidiQ00325.
KOiK15102.
OMAiTIVEQIY.
OrthoDBiEOG7KQ228.
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. Align

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)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti72 – 721G → E in MPCD. 1 Publication
VAR_032850

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei54 – 8330QYSCD…THTAL → YSCEFGSAKYYALCGFGGVL SCGLTHTAV in isoform B. 3 PublicationsVSP_003269Add
BLAST

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
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.3. human. [Q00325-2]
uc001tfo.3. human. [Q00325-1]

Polymorphism databases

DMDMi730052.

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
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 .
CCDSi CCDS9065.1. [Q00325-2 ]
CCDS9066.1. [Q00325-1 ]
PIRi A53737.
B53737.
RefSeqi NP_002626.1. NM_002635.3. [Q00325-2 ]
NP_005879.1. NM_005888.3. [Q00325-1 ]
NP_998776.1. NM_213611.2. [Q00325-2 ]
UniGenei Hs.290404.

3D structure databases

ProteinModelPortali Q00325.
SMRi Q00325. Positions 69-337.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 111269. 72 interactions.
IntActi Q00325. 30 interactions.
MINTi MINT-1156379.
STRINGi 9606.ENSP00000228318.

Protein family/group databases

TCDBi 2.A.29.4.2. the mitochondrial carrier (mc) family.

PTM databases

PhosphoSitei Q00325.

Polymorphism databases

DMDMi 730052.

Proteomic databases

MaxQBi Q00325.
PaxDbi Q00325.
PRIDEi Q00325.

Protocols and materials databases

DNASUi 5250.
Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENST00000188376 ; 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 ]
GeneIDi 5250.
KEGGi hsa:5250.
UCSCi uc001tfm.3. human. [Q00325-2 ]
uc001tfo.3. human. [Q00325-1 ]

Organism-specific databases

CTDi 5250.
GeneCardsi GC12P098997.
HGNCi HGNC:10989. SLC25A3.
HPAi HPA045709.
MIMi 600370. gene.
610773. phenotype.
neXtProti NX_Q00325.
Orphaneti 91130. Cardiomyopathy - hypotonia - lactic acidosis.
PharmGKBi PA35865.
GenAtlasi Search...

Phylogenomic databases

eggNOGi NOG265500.
GeneTreei ENSGT00390000008708.
HOGENOMi HOG000164438.
HOVERGENi HBG024440.
InParanoidi Q00325.
KOi K15102.
OMAi TIVEQIY.
OrthoDBi EOG7KQ228.
PhylomeDBi Q00325.
TreeFami TF314119.

Miscellaneous databases

ChiTaRSi slc25a3. human.
GeneWikii SLC25A3.
GenomeRNAii 5250.
NextBioi 20278.
PROi Q00325.
SOURCEi Search...

Gene expression databases

Bgeei Q00325.
CleanExi HS_SLC25A3.
ExpressionAtlasi Q00325. baseline and differential.
Genevestigatori Q00325.

Family and domain databases

Gene3Di 1.50.40.10. 1 hit.
InterProi IPR018108. Mitochondrial_sb/sol_carrier.
IPR023395. Mt_carrier_dom.
[Graphical view ]
Pfami PF00153. Mito_carr. 3 hits.
[Graphical view ]
SUPFAMi SSF103506. SSF103506. 1 hit.
PROSITEi PS50920. SOLCAR. 3 hits.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "The sequences of human and bovine genes of the phosphate carrier from mitochondria contain evidence of alternatively spliced forms."
    Dolce V., Iacobazzi V., Palmieri F., Walker J.E.
    J. Biol. Chem. 269:10451-10460(1994) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] (ISOFORMS A AND B).
    Tissue: Placenta.
  2. "Nucleotide sequence of a human heart cDNA encoding the mitochondrial phosphate carrier."
    Dolce V., Fiermonte G., Messina A., Palmieri F.
    DNA Seq. 2:133-135(1991) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM B).
    Tissue: Heart.
  3. "Identification of immuno-peptidmics that are recognized by tumor-reactive CTL generated from TIL of colon cancer patients."
    Shichijo S., Itoh K.
    Submitted (JUL-2001) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM B).
    Tissue: Colon adenocarcinoma.
  4. "Complete sequencing and characterization of 21,243 full-length human cDNAs."
    Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.
    , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
    Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM A).
    Tissue: Skeletal muscle.
  5. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  6. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM B).
    Tissue: Brain, Cervix, Lung, Ovary, Placenta and Prostate.
  7. Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT TYR-196, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  8. "Lysine acetylation targets protein complexes and co-regulates major cellular functions."
    Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.C., Olsen J.V., Mann M.
    Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-99, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  9. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  10. Cited for: VARIANT MPCD GLU-72.

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: October 29, 2014
This is version 148 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

External Data

Dasty 3