Skip Header

You are using a version of Internet Explorer that may not display all features of this website. Please upgrade to a modern browser.
Contribute Send feedback
Read comments (?) or add your own

P55916 (UCP3_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 131. 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·Interactions·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Mitochondrial uncoupling protein 3

Short name=UCP 3
Alternative name(s):
Solute carrier family 25 member 9
Gene names
Name:UCP3
Synonyms:SLC25A9
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length312 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

UCP are mitochondrial transporter proteins that create proton leaks across the inner mitochondrial membrane, thus uncoupling oxidative phosphorylation. As a result, energy is dissipated in the form of heat. May play a role in the modulation of tissue respiratory control. Participates in thermogenesis and energy balance.

Subcellular location

Mitochondrion inner membrane; Multi-pass membrane protein By similarity.

Tissue specificity

Only in skeletal muscle and heart. Is more expressed in glycolytic than in oxidative skeletal muscles. Ref.3

Involvement in disease

Obesity (OBESITY) [MIM:601665]: A condition characterized by an increase of body weight beyond the limitation of skeletal and physical requirements, as the result of excessive accumulation of body fat.
Note: The gene represented in this entry may be involved in disease pathogenesis. Ref.8 Ref.9

Sequence similarities

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

Contains 3 Solcar repeats.

Sequence caution

The sequence AAC51785.1 differs from that shown. Reason: Erroneous initiation.

Ontologies

Keywords
   Biological processTransport
   Cellular componentMembrane
Mitochondrion
Mitochondrion inner membrane
   Coding sequence diversityAlternative splicing
Polymorphism
   DiseaseDiabetes mellitus
Disease mutation
Obesity
   DomainRepeat
Transmembrane
Transmembrane helix
   Technical termComplete proteome
Reference proteome
Gene Ontology (GO)
   Biological_processaging

Inferred from electronic annotation. Source: Ensembl

cellular metabolic process

Traceable author statement. Source: Reactome

cellular response to hormone stimulus

Inferred from electronic annotation. Source: Ensembl

fatty acid metabolic process

Inferred from electronic annotation. Source: Ensembl

lipid metabolic process

Traceable author statement PubMed 10935638. Source: ProtInc

mitochondrial transport

Inferred from electronic annotation. Source: InterPro

proton transport

Traceable author statement. Source: Reactome

respiratory electron transport chain

Traceable author statement. Source: Reactome

respiratory gaseous exchange

Traceable author statement Ref.1. Source: ProtInc

response to activity

Inferred from electronic annotation. Source: Ensembl

response to cold

Inferred from electronic annotation. Source: Ensembl

response to glucocorticoid

Inferred from electronic annotation. Source: Ensembl

response to hypoxia

Inferred from electronic annotation. Source: Ensembl

response to insulin

Inferred from electronic annotation. Source: Ensembl

response to nutrient

Inferred from electronic annotation. Source: Ensembl

response to superoxide

Inferred from electronic annotation. Source: Ensembl

small molecule metabolic process

Traceable author statement. Source: Reactome

   Cellular_componentintegral component of membrane

Inferred from electronic annotation. Source: UniProtKB-KW

mitochondrial inner membrane

Traceable author statement. Source: Reactome

mitochondrion

Traceable author statement Ref.1. Source: ProtInc

   Molecular_functionoxidative phosphorylation uncoupler activity

Inferred from electronic annotation. Source: Ensembl

protein binding

Inferred from physical interaction PubMed 24008843. Source: IntAct

transporter activity

Traceable author statement Ref.3. Source: ProtInc

Complete GO annotation...

Binary interactions

With

Entry

#Exp.

IntAct

Notes

NSP034952EBI-9116865,EBI-2548993From a different organism.

Alternative products

This entry describes 3 isoforms produced by alternative splicing. [Align] [Select]
Isoform UCP3L (identifier: P55916-1)

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 UCP3S (identifier: P55916-2)

The sequence of this isoform differs from the canonical sequence as follows:
     276-312: Missing.
Isoform 3 (identifier: P55916-3)

The sequence of this isoform differs from the canonical sequence as follows:
     114-216: Missing.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 312312Mitochondrial uncoupling protein 3
PRO_0000090672

Regions

Transmembrane11 – 3222Helical; Name=1; Potential
Transmembrane77 – 9923Helical; Name=2; Potential
Transmembrane120 – 13617Helical; Name=3; Potential
Transmembrane184 – 20017Helical; Name=4; Potential
Transmembrane218 – 23720Helical; Name=5; Potential
Transmembrane272 – 29423Helical; Name=6; Potential
Repeat11 – 10595Solcar 1
Repeat114 – 20693Solcar 2
Repeat215 – 30086Solcar 3
Region279 – 30123Purine nucleotide binding By similarity

Natural variations

Alternative sequence114 – 216103Missing in isoform 3.
VSP_003270
Alternative sequence276 – 31237Missing in isoform UCP3S.
VSP_003271
Natural variant91V → M.
Corresponds to variant rs8179180 [ dbSNP | Ensembl ].
VAR_050136
Natural variant701R → W in severe obesity with type 2 diabetes. Ref.9
Corresponds to variant rs17848368 [ dbSNP | Ensembl ].
VAR_004407
Natural variant1021V → I in obesity. Ref.8
Corresponds to variant rs2229707 [ dbSNP | Ensembl ].
VAR_004408

Experimental info

Sequence conflict193 – 1942NC → KS in AAC18822. Ref.5

Sequences

Sequence LengthMass (Da)Tools
Isoform UCP3L [UniParc].

Last modified November 1, 1997. Version 1.
Checksum: D0E04A8DB352B17C

FASTA31234,216
        10         20         30         40         50         60 
MVGLKPSDVP PTMAVKFLGA GTAACFADLV TFPLDTAKVR LQIQGENQAV QTARLVQYRG 

        70         80         90        100        110        120 
VLGTILTMVR TEGPCSPYNG LVAGLQRQMS FASIRIGLYD SVKQVYTPKG ADNSSLTTRI 

       130        140        150        160        170        180 
LAGCTTGAMA VTCAQPTDVV KVRFQASIHL GPSRSDRKYS GTMDAYRTIA REEGVRGLWK 

       190        200        210        220        230        240 
GTLPNIMRNA IVNCAEVVTY DILKEKLLDY HLLTDNFPCH FVSAFGAGFC ATVVASPVDV 

       250        260        270        280        290        300 
VKTRYMNSPP GQYFSPLDCM IKMVAQEGPT AFYKGFTPSF LRLGSWNVVM FVTYEQLKRA 

       310 
LMKVQMLRES PF 

« Hide

Isoform UCP3S [UniParc].

Checksum: 4E0FDF29E06A8AD9
Show »

FASTA27529,783
Isoform 3 [UniParc].

Checksum: 4DC46DECA9A50E17
Show »

FASTA20922,874

References

« Hide 'large scale' references
[1]"Uncoupling protein-3: a new member of the mitochondrial carrier family with tissue-specific expression."
Boss O., Samec S., Paoloni-Giacobino A., Dulloo A., Seydoux J., Rossier C., Muzzin P., Giacobino J.-P.
FEBS Lett. 408:39-42(1997) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
Tissue: Skeletal muscle.
[2]"The human uncoupling protein-3 gene. Genomic structure, chromosomal localization, and genetic basis for short and long form transcripts."
Solanes G., Vidal-Puig A., Grujic D., Flier J.S., Lowell B.B.
J. Biol. Chem. 272:25433-25436(1997) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] (ISOFORMS UCP3L AND UCP3S).
[3]"UCP3: an uncoupling protein homologue expressed preferentially and abundantly in skeletal muscle and brown adipose tissue."
Vidal-Puig A., Solanes G., Grujic D., Flier J.S., Lowell B.B.
Biochem. Biophys. Res. Commun. 235:79-82(1997) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM UCP3L), TISSUE SPECIFICITY.
[4]"Uncoupling protein-3 is a mediator of thermogenesis regulated by thyroid hormone, beta3-adrenergic agonists, and leptin."
Gong D.-W., He Y., Karas M., Reitman M.
J. Biol. Chem. 272:24129-24132(1997) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[5]"Organisation of the coding exons and mutational screening of the uncoupling protein 3 gene in subjects with juvenile-onset obesity."
Urhammer S.A., Dalgaard L.T., Soerensen T.I.A., Tybjaerg-Hansen A., Echwald S.M., Andersen T., Clausen J.O., Pedersen O.
Diabetologia 41:241-244(1998) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[6]"The uncoupling protein-3 gene is transcribed from tissue-specific promoters in humans but not in rodents."
Esterbauer H., Oberkofler H., Krempler F., Strosberg A.D., Patsch W.
J. Biol. Chem. 275:36394-36399(2000) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[7]"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 3).
Tissue: Skeletal muscle.
[8]"Effects of mutations in the human uncoupling protein 3 gene on the respiratory quotient and fat oxidation in severe obesity and type 2 diabetes."
Argyropoulos G., Brown A.M., Willi S.M., Zhu J., He Y., Reitman M., Gevao S.M., Spruill I., Garvey W.T.
J. Clin. Invest. 102:1345-1351(1998) [PubMed] [Europe PMC] [Abstract]
Cited for: VARIANT OBESITY ILE-102.
[9]"A novel missense mutation, R70W, in the human uncoupling protein 3 gene in a family with type 2 diabetes."
Brown A.M., Willi S.M., Argyropoulos G., Garvey W.T.
Hum. Mutat. 13:506-506(1999)
Cited for: VARIANT OBESITY TRP-70.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
U84763 mRNA. Translation: AAC51367.1.
U82818 mRNA. Translation: AAC51356.1.
AF001787 mRNA. Translation: AAC51369.1.
AF011449 mRNA. Translation: AAC51767.1.
AF012202 expand/collapse EMBL AC list , AF012197, AF012198, AF012199, AF012200, AF012201 Genomic DNA. Translation: AAC51785.1. Different initiation.
AF026958, AF026956, AF026957 Genomic DNA. Translation: AAC18822.1.
AF050113 Genomic DNA. Translation: AAG02284.1.
BC008392 mRNA. Translation: AAH08392.1.
CCDSCCDS44677.1. [P55916-2]
CCDS8229.1. [P55916-1]
PIRJC5522.
RefSeqNP_003347.1. NM_003356.3. [P55916-1]
NP_073714.1. NM_022803.2. [P55916-2]
UniGeneHs.101337.
Hs.621879.

3D structure databases

ProteinModelPortalP55916.
SMRP55916. Positions 13-311.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid113199. 3 interactions.
IntActP55916. 1 interaction.
STRING9606.ENSP00000323740.

Protein family/group databases

TCDB2.A.29.3.5. the mitochondrial carrier (mc) family.

Proteomic databases

PaxDbP55916.
PRIDEP55916.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000314032; ENSP00000323740; ENSG00000175564. [P55916-1]
ENST00000426995; ENSP00000392143; ENSG00000175564. [P55916-2]
GeneID7352.
KEGGhsa:7352.
UCSCuc001our.3. human. [P55916-1]

Organism-specific databases

CTD7352.
GeneCardsGC11M073711.
HGNCHGNC:12519. UCP3.
HPACAB010771.
MIM601665. phenotype.
602044. gene.
neXtProtNX_P55916.
PharmGKBPA37166.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG300436.
HOGENOMHOG000165140.
HOVERGENHBG009528.
InParanoidP55916.
KOK15103.
OMARFQASIH.
OrthoDBEOG793B7Z.
PhylomeDBP55916.
TreeFamTF323211.

Enzyme and pathway databases

ReactomeREACT_111217. Metabolism.

Gene expression databases

ArrayExpressP55916.
BgeeP55916.
CleanExHS_UCP3.
GenevestigatorP55916.

Family and domain databases

Gene3D1.50.40.10. 1 hit.
InterProIPR002030. Mit_uncoupling.
IPR018108. Mitochondrial_sb/sol_carrier.
IPR023395. Mt_carrier_dom.
[Graphical view]
PfamPF00153. Mito_carr. 3 hits.
[Graphical view]
PRINTSPR00784. MTUNCOUPLING.
SUPFAMSSF103506. SSF103506. 1 hit.
PROSITEPS50920. SOLCAR. 3 hits.
[Graphical view]
ProtoNetSearch...

Other

GeneWikiUCP3.
GenomeRNAi7352.
NextBio28782.
PROP55916.
SOURCESearch...

Entry information

Entry nameUCP3_HUMAN
AccessionPrimary (citable) accession number: P55916
Secondary accession number(s): O60475, Q96HL3
Entry history
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: November 1, 1997
Last modified: July 9, 2014
This is version 131 of the entry and version 1 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 11

Human chromosome 11: entries, gene names and cross-references to MIM