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Q00325

- MPCP_HUMAN

UniProt

Q00325 - MPCP_HUMAN

Protein

Phosphate carrier protein, mitochondrial

Gene

SLC25A3

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 147 (01 Oct 2014)
      Sequence version 2 (01 Feb 1995)
      Previous versions | rss
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    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

    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

    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

    ArrayExpressiQ00325.
    BgeeiQ00325.
    CleanExiHS_SLC25A3.
    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.
    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

    MFSSVAHLAR ANPFNTPHLQ LVHDGLGDLR SSSPGPTGQP RRPRNLAAAA    50
    VEEQYSCDYG SGRFFILCGL GGIISCGTTH TALVPLDLVK CRMQVDPQKY 100
    KGIFNGFSVT LKEDGVRGLA KGWAPTFLGY SMQGLCKFGF YEVFKVLYSN 150
    MLGEENTYLW RTSLYLAASA SAEFFADIAL APMEAAKVRI QTQPGYANTL 200
    RDAAPKMYKE EGLKAFYKGV APLWMRQIPY TMMKFACFER TVEALYKFVV 250
    PKPRSECSKP EQLVVTFVAG YIAGVFCAIV SHPADSVVSV LNKEKGSSAS 300
    LVLKRLGFKG VWKGLFARII MIGTLTALQW FIYDSVKVYF RLPRPPPPEM 350
    PESLKKKLGL TQ 362
    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 GC12P098920.
    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.
    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

    ArrayExpressi Q00325.
    Bgeei Q00325.
    CleanExi HS_SLC25A3.
    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

    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 1, 2014
    This is version 147 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