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Q00325 (MPCP_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 142. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (5) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Phosphate carrier protein, mitochondrial
Alternative name(s):
Phosphate transport protein
Short name=PTP
Solute carrier family 25 member 3
Gene names
Name:SLC25A3
Synonyms:PHC
ORF Names:OK/SW-cl.48
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length362 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

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).

Subunit structure

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

Subcellular location

Mitochondrion inner membrane; Multi-pass membrane protein.

Involvement in disease

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.
Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.10

Sequence similarities

Belongs to the mitochondrial carrier (TC 2.A.29) family. [View classification]

Contains 3 Solcar repeats.

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]
Isoform A (identifier: Q00325-1)

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.
Isoform B (identifier: Q00325-2)

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

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 4949Mitochondrion By similarity
Chain50 – 362313Phosphate carrier protein, mitochondrial
PRO_0000019256

Regions

Topological domain50 – 6314Mitochondrial intermembrane Potential
Transmembrane64 – 8623Helical; Name=1; Potential
Topological domain87 – 12135Mitochondrial matrix Potential
Transmembrane122 – 14120Helical; Name=2; Potential
Topological domain142 – 16120Mitochondrial intermembrane Potential
Transmembrane162 – 18322Helical; Name=3; Potential
Topological domain184 – 21835Mitochondrial matrix Potential
Transmembrane219 – 23820Helical; Name=4; Potential
Topological domain239 – 26123Mitochondrial intermembrane Potential
Transmembrane262 – 28423Helical; Name=5; Potential
Topological domain285 – 31430Mitochondrial matrix Potential
Transmembrane315 – 33319Helical; Name=6; Potential
Topological domain334 – 36229Mitochondrial intermembrane Potential
Repeat63 – 14785Solcar 1
Repeat160 – 24485Solcar 2
Repeat261 – 33979Solcar 3

Amino acid modifications

Modified residue991N6-acetyllysine Ref.8
Modified residue1961Phosphotyrosine Ref.7
Modified residue2091N6-acetyllysine By similarity

Natural variations

Alternative sequence54 – 8330QYSCD…THTAL → YSCEFGSAKYYALCGFGGVL SCGLTHTAV in isoform B.
VSP_003269
Natural variant721G → E in MPCD. Ref.10
VAR_032850

Sequences

Sequence LengthMass (Da)Tools
Isoform A [UniParc].

Last modified February 1, 1995. Version 2.
Checksum: 78714C85931B22D5

FASTA36240,095
        10         20         30         40         50         60 
MFSSVAHLAR ANPFNTPHLQ LVHDGLGDLR SSSPGPTGQP RRPRNLAAAA VEEQYSCDYG 

        70         80         90        100        110        120 
SGRFFILCGL GGIISCGTTH TALVPLDLVK CRMQVDPQKY KGIFNGFSVT LKEDGVRGLA 

       130        140        150        160        170        180 
KGWAPTFLGY SMQGLCKFGF YEVFKVLYSN MLGEENTYLW RTSLYLAASA SAEFFADIAL 

       190        200        210        220        230        240 
APMEAAKVRI QTQPGYANTL RDAAPKMYKE EGLKAFYKGV APLWMRQIPY TMMKFACFER 

       250        260        270        280        290        300 
TVEALYKFVV PKPRSECSKP EQLVVTFVAG YIAGVFCAIV SHPADSVVSV LNKEKGSSAS 

       310        320        330        340        350        360 
LVLKRLGFKG VWKGLFARII MIGTLTALQW FIYDSVKVYF RLPRPPPPEM PESLKKKLGL 


TQ 

« Hide

Isoform B [UniParc].

Checksum: 43AD380B15553DA6
Show »

FASTA36139,959

References

« Hide 'large scale' references
[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. expand/collapse author list , 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]Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. expand/collapse author list , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C.
Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases
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]"Large-scale proteomics analysis of the human kinome."
Oppermann F.S., Gnad F., Olsen J.V., Hornberger R., Greff Z., Keri G., Mann M., Daub H.
Mol. Cell. Proteomics 8:1751-1764(2009) [PubMed] [Europe PMC] [Abstract]
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]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
[10]"Mitochondrial phosphate-carrier deficiency: a novel disorder of oxidative phosphorylation."
Mayr J.A., Merkel O., Kohlwein S.D., Gebhardt B.R., Boehles H., Foetschl U., Koch J., Jaksch M., Lochmueller H., Horvath R., Freisinger P., Sperl W.
Am. J. Hum. Genet. 80:478-484(2007) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT MPCD GLU-72.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
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.
PIRA53737.
B53737.
RefSeqNP_002626.1. NM_002635.3.
NP_005879.1. NM_005888.3.
NP_998776.1. NM_213611.2.
UniGeneHs.290404.

3D structure databases

ProteinModelPortalQ00325.
SMRQ00325. Positions 69-337.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid111269. 82 interactions.
IntActQ00325. 30 interactions.
MINTMINT-1156379.
STRING9606.ENSP00000228318.

Protein family/group databases

TCDB2.A.29.4.2. the mitochondrial carrier (mc) family.

PTM databases

PhosphoSiteQ00325.

Polymorphism databases

DMDM730052.

Proteomic databases

PaxDbQ00325.
PRIDEQ00325.

Protocols and materials databases

DNASU5250.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000188376; 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]
GeneID5250.
KEGGhsa:5250.
UCSCuc001tfm.3. human. [Q00325-2]
uc001tfo.3. human. [Q00325-1]

Organism-specific databases

CTD5250.
GeneCardsGC12P098920.
HGNCHGNC:10989. SLC25A3.
HPAHPA045709.
MIM600370. gene.
610773. phenotype.
neXtProtNX_Q00325.
Orphanet91130. Cardiomyopathy - hypotonia - lactic acidosis.
PharmGKBPA35865.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG265500.
HOGENOMHOG000164438.
HOVERGENHBG024440.
InParanoidQ00325.
KOK15102.
OMAKERQLPF.
OrthoDBEOG7KQ228.
PhylomeDBQ00325.
TreeFamTF314119.

Gene expression databases

ArrayExpressQ00325.
BgeeQ00325.
CleanExHS_SLC25A3.
GenevestigatorQ00325.

Family and domain databases

Gene3D1.50.40.10. 1 hit.
InterProIPR018108. Mitochondrial_sb/sol_carrier.
IPR023395. Mt_carrier_dom.
[Graphical view]
PfamPF00153. Mito_carr. 3 hits.
[Graphical view]
SUPFAMSSF103506. SSF103506. 1 hit.
PROSITEPS50920. SOLCAR. 3 hits.
[Graphical view]
ProtoNetSearch...

Other

ChiTaRSslc25a3. human.
GeneWikiSLC25A3.
GenomeRNAi5250.
NextBio20278.
PROQ00325.
SOURCESearch...

Entry information

Entry nameMPCP_HUMAN
AccessionPrimary (citable) accession number: Q00325
Secondary accession number(s): B3KS34, Q7Z7N7, Q96A03
Entry history
Integrated into UniProtKB/Swiss-Prot: December 1, 1992
Last sequence update: February 1, 1995
Last modified: April 16, 2014
This is version 142 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome 12

Human chromosome 12: entries, gene names and cross-references to MIM