UCP3

Uncoupling protein 3 (mitochondrial, proton carrier)

Identifiers

UCP3 ; SLC25A9

External IDs

OMIM: 602044 MGI: 1099787 HomoloGene: 2517 GeneCards: UCP3 Gene

Gene ontology
Molecular function	• transporter activity • protein binding • oxidative phosphorylation uncoupler activity
Cellular component	• mitochondrion • mitochondrial inner membrane • integral component of membrane
Biological process	• response to superoxide • response to hypoxia • lipid metabolic process • fatty acid metabolic process • mitochondrial transport • aging • response to nutrient • respiratory gaseous exchange • response to cold • response to activity • proton transport • respiratory electron transport chain • response to insulin • cellular response to hormone stimulus • cellular metabolic process • small molecule metabolic process • response to glucocorticoid
Sources: Amigo / QuickGO

RNA expression pattern

More reference expression data

Orthologs

Species

Human

Mouse

RefSeq (mRNA)

RefSeq (protein)

Location (UCSC)

Chr 11:
74 – 74.01 Mb

Chr 7:
100.47 – 100.49 Mb

PubMed search

Mitochondrial uncoupling protein 3 is a protein that in humans is encoded by the UCP3 gene.^[1]^[2]

Function

Mitochondrial uncoupling proteins (UCP) are members of the larger family of mitochondrial anion carrier proteins (MACP). UCPs separate oxidative phosphorylation from ATP synthesis with energy dissipated as heat, also referred to as the mitochondrial proton leak. UCPs facilitate the transfer of anions from the inner to the outer mitochondrial membrane and the return transfer of protons from the outer to the inner mitochondrial membrane. They also reduce the mitochondrial membrane potential in mammalian cells. Tissue specificity occurs for the different UCPs and the exact methods of how UCPs transfer H⁺/OH⁻ are not known.^[3]

Structure

UCPs contain the three homologous protein domains of MACPs.^[3]

Gene regulation

This gene has tissue-specific transcription initiation with other transcription initiation sites upstream of SM-1 (major skeletal muscle site). Chromosomal order is 5'-UCP3-UCP2-3'. Two splice variants have been found for this gene.^[3]

Disease association

Mutations in the UCP3 gene are associated with obesity.^[4]^[5]

Interactions

UCP3 has been shown to interact with YWHAQ.^[6]

References

↑ Boss O, Giacobino JP, Muzzin P (April 1998). "Genomic structure of uncoupling protein-3 (UCP3) and its assignment to chromosome 11q13". Genomics 47 (3): 425–6. doi:10.1006/geno.1997.5135. PMID 9480760.
↑ Vidal-Puig A, Solanes G, Grujic D, Flier JS, Lowell BB (July 1997). "UCP3: an uncoupling protein homologue expressed preferentially and abundantly in skeletal muscle and brown adipose tissue". Biochem Biophys Res Commun 235 (1): 79–82. doi:10.1006/bbrc.1997.6740. PMID 9196039.
1 2 3 "Entrez Gene: UCP3 uncoupling protein 3 (mitochondrial, proton carrier)".
↑ Argyropoulos G, Brown AM, Willi SM, Zhu J, He Y, Reitman M, Gevao SM, Spruill I, Garvey WT (October 1998). "Effects of mutations in the human uncoupling protein 3 gene on the respiratory quotient and fat oxidation in severe obesity and type 2 diabetes". J. Clin. Invest. 102 (7): 1345–51. doi:10.1172/JCI4115. PMC 508981. PMID 9769326.
↑ Dalgaard LT, Sørensen TI, Drivsholm T, Borch-Johnsen K, Andersen T, Hansen T, Pedersen O (March 2001). "A prevalent polymorphism in the promoter of the UCP3 gene and its relationship to body mass index and long term body weight change in the Danish population". J. Clin. Endocrinol. Metab. 86 (3): 1398–402. doi:10.1210/jc.86.3.1398. PMID 11238538.
↑ Pierrat B, Ito M, Hinz W, Simonen M, Erdmann D, Chiesi M, Heim J (May 2000). "Uncoupling proteins 2 and 3 interact with members of the 14.3.3 family". Eur. J. Biochem. 267 (9): 2680–7. doi:10.1046/j.1432-1327.2000.01285.x. PMID 10785390.

Ricquier D, Bouillaud F (2000). "The uncoupling protein homologues: UCP1, UCP2, UCP3, StUCP and AtUCP". Biochem. J. 345 (2): 161–79. doi:10.1042/0264-6021:3450161. PMC 1220743. PMID 10620491.
Muzzin P (2002). "The uncoupling proteins". Ann. Endocrinol. (Paris) 63 (2 Pt 1): 106–10. PMID 11994670.
Boss O, Samec S, Paoloni-Giacobino A, Rossier C, Dulloo A, Seydoux J, Muzzin P, Giacobino JP (1997). "Uncoupling protein-3: a new member of the mitochondrial carrier family with tissue-specific expression". FEBS Lett. 408 (1): 39–42. doi:10.1016/S0014-5793(97)00384-0. PMID 9180264.
Gong DW, He Y, Karas M, Reitman M (1997). "Uncoupling protein-3 is a mediator of thermogenesis regulated by thyroid hormone, beta3-adrenergic agonists, and leptin". J. Biol. Chem. 272 (39): 24129–32. doi:10.1074/jbc.272.39.24129. PMID 9305858.
Solanes G, Vidal-Puig A, Grujic D, Flier JS, Lowell BB (1997). "The human uncoupling protein-3 gene. Genomic structure, chromosomal localization, and genetic basis for short and long form transcripts". J. Biol. Chem. 272 (41): 25433–6. doi:10.1074/jbc.272.41.25433. PMID 9325252.
Urhammer SA, Dalgaard LT, Sørensen TI, Tybjaerg-Hansen A, Echwald SM, Andersen T, Clausen JO, Pedersen O (1998). "Organisation of the coding exons and mutational screening of the uncoupling protein 3 gene in subjects with juvenile-onset obesity". Diabetologia 41 (2): 241–4. doi:10.1007/s001250050897. PMID 9498661.
Argyropoulos G, Brown AM, Willi SM, Zhu J, He Y, Reitman M, Gevao SM, Spruill I, Garvey WT (1998). "Effects of mutations in the human uncoupling protein 3 gene on the respiratory quotient and fat oxidation in severe obesity and type 2 diabetes". J. Clin. Invest. 102 (7): 1345–51. doi:10.1172/JCI4115. PMC 508981. PMID 9769326.
Acín A, Rodriguez M, Rique H, Canet E, Boutin JA, Galizzi JP (1999). "Cloning and characterization of the 5' flanking region of the human uncoupling protein 3 (UCP3) gene". Biochem. Biophys. Res. Commun. 258 (2): 278–83. doi:10.1006/bbrc.1999.0530. PMID 10329378.
Vidal-Puig AJ, Grujic D, Zhang CY, Hagen T, Boss O, Ido Y, Szczepanik A, Wade J, Mootha V, Cortright R, Muoio DM, Lowell BB (2000). "Energy metabolism in uncoupling protein 3 gene knockout mice". J. Biol. Chem. 275 (21): 16258–66. doi:10.1074/jbc.M910179199. PMID 10748196.
Jezek P, Urbánková E (2000). "Specific sequence of motifs of mitochondrial uncoupling proteins". IUBMB Life 49 (1): 63–70. doi:10.1080/713803586. PMID 10772343.
Pierrat B, Ito M, Hinz W, Simonen M, Erdmann D, Chiesi M, Heim J (2000). "Uncoupling proteins 2 and 3 interact with members of the 14.3.3 family". Eur. J. Biochem. 267 (9): 2680–7. doi:10.1046/j.1432-1327.2000.01285.x. PMID 10785390.
Clapham JC, Arch JR, Chapman H, Haynes A, Lister C, Moore GB, Piercy V, Carter SA, Lehner I, Smith SA, Beeley LJ, Godden RJ, Herrity N, Skehel M, Changani KK, Hockings PD, Reid DG, Squires SM, Hatcher J, Trail B, Latcham J, Rastan S, Harper AJ, Cadenas S, Buckingham JA, Brand MD, Abuin A (2000). "Mice overexpressing human uncoupling protein-3 in skeletal muscle are hyperphagic and lean". Nature 406 (6794): 415–8. Bibcode:2000Natur.406..415C. doi:10.1038/35019082. PMID 10935638.
Esterbauer H, Oberkofler H, Krempler F, Strosberg AD, Patsch W (2000). "The uncoupling protein-3 gene is transcribed from tissue-specific promoters in humans but not in rodents". J. Biol. Chem. 275 (46): 36394–9. doi:10.1074/jbc.M005713200. PMID 10958796.
Lanouette CM, Chagnon YC, Rice T, Pérusse L, Muzzin P, Giacobino JP, Gagnon J, Wilmore JH, Leon AS, Skinner JS, Rao DC, Bouchard C (2002). "Uncoupling protein 3 gene is associated with body composition changes with training in HERITAGE study". J. Appl. Physiol. 92 (3): 1111–8. doi:10.1152/japplphysiol.00726.2001 (inactive 2015-01-09). PMID 11842047.
Collins P, Bing C, McCulloch P, Williams G (2002). "Muscle UCP-3 mRNA levels are elevated in weight loss associated with gastrointestinal adenocarcinoma in humans". Br. J. Cancer 86 (3): 372–5. doi:10.1038/sj.bjc.6600074. PMC 2375209. PMID 11875702.
Hu X, Murphy F, Karwautz A, Li T, Freeman B, Franklin D, Giotakis O, Treasure J, Collier DA (2002). "Analysis of microsatellite markers at the UCP2/UCP3 locus on chromosome 11q13 in anorexia nervosa". Mol. Psychiatry 7 (3): 276–7. doi:10.1038/sj.mp.4001044. PMID 11920154.

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