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Protein

Mitochondrial pyruvate carrier 1

Gene

MPC1

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

Functioni

Mediates the uptake of pyruvate into mitochondria.1 Publication

GO - Molecular functioni

  1. pyruvate transmembrane transporter activity Source: Ensembl

GO - Biological processi

  1. cellular metabolic process Source: Reactome
  2. mitochondrial pyruvate transport Source: Ensembl
  3. pyruvate metabolic process Source: Reactome
  4. small molecule metabolic process Source: Reactome
Complete GO annotation...

Keywords - Biological processi

Transport

Enzyme and pathway databases

ReactomeiREACT_2071. Pyruvate metabolism.

Protein family/group databases

TCDBi2.A.105.1.2. the mitochondrial pyruvate carrier (mpc) family.

Names & Taxonomyi

Protein namesi
Recommended name:
Mitochondrial pyruvate carrier 1
Alternative name(s):
Brain protein 44-like protein
Gene namesi
Name:MPC1
Synonyms:BRP44L
ORF Names:CGI-129, HSPC040, PNAS-115
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componenti: Chromosome 6

Organism-specific databases

HGNCiHGNC:21606. MPC1.

Subcellular locationi

Mitochondrion inner membrane 1 Publication; Multi-pass membrane protein 1 Publication

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transmembranei21 – 4121HelicalSequence AnalysisAdd
BLAST
Transmembranei53 – 7119HelicalSequence AnalysisAdd
BLAST

GO - Cellular componenti

  1. integral component of membrane Source: UniProtKB-KW
  2. mitochondrial inner membrane Source: UniProtKB-SubCell
  3. mitochondrion Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Mitochondrial pyruvate carrier deficiency (MPYCD)1 Publication

The disease is caused by mutations affecting the gene represented in this entry.

Disease descriptionAn autosomal recessive metabolic disorder characterized by severely delayed psychomotor development, mild dysmorphic features, hepatomegaly, marked metabolic acidosis, hyperlactacidemia with normal lactate/pyruvate, and encephalopathy. Some patients have epilepsy and peripheral neuropathy.

See also OMIM:614741
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti79 – 791L → H in MPYCD. 1 Publication
VAR_068099
Natural varianti97 – 971R → W in MPYCD; inactive. 1 Publication
VAR_068100

Keywords - Diseasei

Disease mutation

Organism-specific databases

MIMi614741. phenotype.
PharmGKBiPA134940737.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Initiator methioninei1 – 11RemovedBy similarity
Chaini2 – 109108Mitochondrial pyruvate carrier 1PRO_0000212797Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei2 – 21N-acetylalanineBy similarity
Modified residuei72 – 721N6-acetyllysineBy similarity

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiQ9Y5U8.
PaxDbiQ9Y5U8.
PRIDEiQ9Y5U8.

PTM databases

PhosphoSiteiQ9Y5U8.

Expressioni

Gene expression databases

BgeeiQ9Y5U8.
CleanExiHS_BRP44L.
ExpressionAtlasiQ9Y5U8. baseline and differential.
GenevestigatoriQ9Y5U8.

Organism-specific databases

HPAiHPA045119.
HPA055790.

Interactioni

Subunit structurei

The functional 150 kDa pyruvate import complex is a heteromer of MPC1 and MPC2.By similarity

Protein-protein interaction databases

BioGridi119665. 2 interactions.
IntActiQ9Y5U8. 3 interactions.
STRINGi9606.ENSP00000354223.

Structurei

3D structure databases

ProteinModelPortaliQ9Y5U8.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiNOG288673.
GeneTreeiENSGT00510000046988.
HOGENOMiHOG000188911.
HOVERGENiHBG050728.
InParanoidiQ9Y5U8.
OMAiTTASKEW.
OrthoDBiEOG7DVDF6.
PhylomeDBiQ9Y5U8.
TreeFamiTF314444.

Family and domain databases

InterProiIPR005336. MPC.
[Graphical view]
PfamiPF03650. MPC. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

Q9Y5U8-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAGALVRKAA DYVRSKDFRD YLMSTHFWGP VANWGLPIAA INDMKKSPEI
60 70 80 90 100
ISGRMTFALC CYSLTFMRFA YKVQPRNWLL FACHATNEVA QLIQGGRLIK

HEMTKTASA
Length:109
Mass (Da):12,347
Last modified:November 1, 1999 - v1
Checksum:iE33344CD1AA29124
GO

Sequence cautioni

The sequence AAG23822.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti14 – 141R → Q in AAD34124 (PubMed:10810093).Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti36 – 361L → I.
Corresponds to variant rs11557064 [ dbSNP | Ensembl ].
VAR_052486
Natural varianti79 – 791L → H in MPYCD. 1 Publication
VAR_068099
Natural varianti97 – 971R → W in MPYCD; inactive. 1 Publication
VAR_068100

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF125101 mRNA. Translation: AAD39918.1.
AF151887 mRNA. Translation: AAD34124.1.
AK312201 mRNA. Translation: BAG35134.1.
AL022069 Genomic DNA. Translation: CAI19654.1.
BC000810 mRNA. Translation: AAH00810.1.
AF275811 mRNA. Translation: AAG23822.1. Different initiation.
CCDSiCCDS5293.1.
RefSeqiNP_057182.1. NM_016098.3.
UniGeneiHs.172755.

Genome annotation databases

EnsembliENST00000360961; ENSP00000354223; ENSG00000060762.
GeneIDi51660.
KEGGihsa:51660.
UCSCiuc021zif.2. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF125101 mRNA. Translation: AAD39918.1.
AF151887 mRNA. Translation: AAD34124.1.
AK312201 mRNA. Translation: BAG35134.1.
AL022069 Genomic DNA. Translation: CAI19654.1.
BC000810 mRNA. Translation: AAH00810.1.
AF275811 mRNA. Translation: AAG23822.1. Different initiation.
CCDSiCCDS5293.1.
RefSeqiNP_057182.1. NM_016098.3.
UniGeneiHs.172755.

3D structure databases

ProteinModelPortaliQ9Y5U8.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi119665. 2 interactions.
IntActiQ9Y5U8. 3 interactions.
STRINGi9606.ENSP00000354223.

Protein family/group databases

TCDBi2.A.105.1.2. the mitochondrial pyruvate carrier (mpc) family.

PTM databases

PhosphoSiteiQ9Y5U8.

Proteomic databases

MaxQBiQ9Y5U8.
PaxDbiQ9Y5U8.
PRIDEiQ9Y5U8.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000360961; ENSP00000354223; ENSG00000060762.
GeneIDi51660.
KEGGihsa:51660.
UCSCiuc021zif.2. human.

Organism-specific databases

CTDi51660.
GeneCardsiGC06M166778.
HGNCiHGNC:21606. MPC1.
HPAiHPA045119.
HPA055790.
MIMi614738. gene.
614741. phenotype.
neXtProtiNX_Q9Y5U8.
PharmGKBiPA134940737.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiNOG288673.
GeneTreeiENSGT00510000046988.
HOGENOMiHOG000188911.
HOVERGENiHBG050728.
InParanoidiQ9Y5U8.
OMAiTTASKEW.
OrthoDBiEOG7DVDF6.
PhylomeDBiQ9Y5U8.
TreeFamiTF314444.

Enzyme and pathway databases

ReactomeiREACT_2071. Pyruvate metabolism.

Miscellaneous databases

GenomeRNAii51660.
NextBioi55638.
PROiQ9Y5U8.
SOURCEiSearch...

Gene expression databases

BgeeiQ9Y5U8.
CleanExiHS_BRP44L.
ExpressionAtlasiQ9Y5U8. baseline and differential.
GenevestigatoriQ9Y5U8.

Family and domain databases

InterProiIPR005336. MPC.
[Graphical view]
PfamiPF03650. MPC. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Cloning and functional analysis of cDNAs with open reading frames for 300 previously undefined genes expressed in CD34+ hematopoietic stem/progenitor cells."
    Zhang Q.-H., Ye M., Wu X.-Y., Ren S.-X., Zhao M., Zhao C.-J., Fu G., Shen Y., Fan H.-Y., Lu G., Zhong M., Xu X.-R., Han Z.-G., Zhang J.-W., Tao J., Huang Q.-H., Zhou J., Hu G.-X.
    , Gu J., Chen S.-J., Chen Z.
    Genome Res. 10:1546-1560(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Umbilical cord blood.
  2. "Identification of novel human genes evolutionarily conserved in Caenorhabditis elegans by comparative proteomics."
    Lai C.-H., Chou C.-Y., Ch'ang L.-Y., Liu C.-S., Lin W.-C.
    Genome Res. 10:703-713(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
  3. "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(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Skeletal muscle.
  4. "The DNA sequence and analysis of human chromosome 6."
    Mungall A.J., Palmer S.A., Sims S.K., Edwards C.A., Ashurst J.L., Wilming L., Jones M.C., Horton R., Hunt S.E., Scott C.E., Gilbert J.G.R., Clamp M.E., Bethel G., Milne S., Ainscough R., Almeida J.P., Ambrose K.D., Andrews T.D.
    , Ashwell R.I.S., Babbage A.K., Bagguley C.L., Bailey J., Banerjee R., Barker D.J., Barlow K.F., Bates K., Beare D.M., Beasley H., Beasley O., Bird C.P., Blakey S.E., Bray-Allen S., Brook J., Brown A.J., Brown J.Y., Burford D.C., Burrill W., Burton J., Carder C., Carter N.P., Chapman J.C., Clark S.Y., Clark G., Clee C.M., Clegg S., Cobley V., Collier R.E., Collins J.E., Colman L.K., Corby N.R., Coville G.J., Culley K.M., Dhami P., Davies J., Dunn M., Earthrowl M.E., Ellington A.E., Evans K.A., Faulkner L., Francis M.D., Frankish A., Frankland J., French L., Garner P., Garnett J., Ghori M.J., Gilby L.M., Gillson C.J., Glithero R.J., Grafham D.V., Grant M., Gribble S., Griffiths C., Griffiths M.N.D., Hall R., Halls K.S., Hammond S., Harley J.L., Hart E.A., Heath P.D., Heathcott R., Holmes S.J., Howden P.J., Howe K.L., Howell G.R., Huckle E., Humphray S.J., Humphries M.D., Hunt A.R., Johnson C.M., Joy A.A., Kay M., Keenan S.J., Kimberley A.M., King A., Laird G.K., Langford C., Lawlor S., Leongamornlert D.A., Leversha M., Lloyd C.R., Lloyd D.M., Loveland J.E., Lovell J., Martin S., Mashreghi-Mohammadi M., Maslen G.L., Matthews L., McCann O.T., McLaren S.J., McLay K., McMurray A., Moore M.J.F., Mullikin J.C., Niblett D., Nickerson T., Novik K.L., Oliver K., Overton-Larty E.K., Parker A., Patel R., Pearce A.V., Peck A.I., Phillimore B.J.C.T., Phillips S., Plumb R.W., Porter K.M., Ramsey Y., Ranby S.A., Rice C.M., Ross M.T., Searle S.M., Sehra H.K., Sheridan E., Skuce C.D., Smith S., Smith M., Spraggon L., Squares S.L., Steward C.A., Sycamore N., Tamlyn-Hall G., Tester J., Theaker A.J., Thomas D.W., Thorpe A., Tracey A., Tromans A., Tubby B., Wall M., Wallis J.M., West A.P., White S.S., Whitehead S.L., Whittaker H., Wild A., Willey D.J., Wilmer T.E., Wood J.M., Wray P.W., Wyatt J.C., Young L., Younger R.M., Bentley D.R., Coulson A., Durbin R.M., Hubbard T., Sulston J.E., Dunham I., Rogers J., Beck S.
    Nature 425:805-811(2002) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  5. "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(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Placenta.
  6. "Human acute promyelocytic leukemia cell line NB4's apoptosis related genes."
    Yu W.-Q., Chai Y.-B., Sun B.-Z., Zhu F., Liu X.-S., Li Z., Lu F., Yan W., Yang H., Zhao Z.-L.
    Submitted (MAY-2000) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 53-109.
    Tissue: Promyelocytic leukemia.
  7. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  8. Cited for: FUNCTION, SUBCELLULAR LOCATION, VARIANTS MPYCD HIS-79 AND TRP-97.
  9. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
    Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.
    J. Proteomics 96:253-262(2013) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

Entry informationi

Entry nameiMPC1_HUMAN
AccessioniPrimary (citable) accession number: Q9Y5U8
Secondary accession number(s): B2R5I7
, Q5TI66, Q9HB67, Q9UQN4
Entry historyi
Integrated into UniProtKB/Swiss-Prot: September 19, 2002
Last sequence update: November 1, 1999
Last modified: March 4, 2015
This is version 106 of the entry and version 1 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 6
    Human chromosome 6: 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

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 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.