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31 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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UCP3
uncoupling protein 3
Chromosome 11 · 11q13.4
NCBI Gene: 7352Ensembl: ENSG00000175564.14HGNC: HGNC:12519UniProt: A0A0S2Z4G5
195PubMed Papers
21Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
calcium ion transmembrane transporter activityproton transmembrane transporter activityoxidative phosphorylation uncoupler activityproton transmembrane transportmorbid obesityobesityinherited obesityMODY
✦AI Summary

UCP3 (uncoupling protein 3) is a mitochondrial transmembrane transporter predominantly expressed in skeletal muscle that plays a multifaceted role in energy metabolism 1. Originally characterized as a proton leak channel that uncouples oxidative phosphorylation from ATP synthesis 2, UCP3's primary function remains controversial. Emerging evidence suggests uncoupling may be a secondary consequence rather than the primary function, with UCP3 potentially serving as a metabolite transporter, particularly for fatty acids or lipid peroxides 34. UCP3 critically regulates substrate utilization by favoring lipid oxidation during high-fat conditions and fasting, while also promoting glucose uptake and oxidation 1. The protein exhibits inducible proton transport activity activated by fatty acids, thyroid hormones, and catecholamines 1. Additional proposed functions include attenuating reactive oxygen species (ROS) production and protecting against oxidative damage 4. UCP3 is upregulated during fasting to enhance lipid utilization while simultaneously suppressing uncoupling activity, optimizing whole-body energy homeostasis 1. Clinically, UCP3 variants have been associated with obesity and severe early-onset obesity, particularly when co-occurring with variants in other metabolic genes like PCSK1 56. UCP3 remains a promising therapeutic target for metabolic disorders, though its precise mechanistic role requires further investigation.

Sources cited
1
UCP3 regulates substrate utilization favoring lipid oxidation, promotes glucose uptake, exhibits inducible proton transport and uncoupling activity, and is upregulated during fasting
PMID: 38499130
2
UCP3 can uncouple ATP production from mitochondrial respiration but its primary function is not energy metabolism regulation; putative roles include ROS regulation and fatty acid transport
PMID: 12110661
3
UCP3 induction in humans does not result in apparent mitochondrial uncoupling; primary function may involve exporting fatty acid anions and lipid peroxides from mitochondrial matrix
PMID: 16175492
4
UCP3 catalyzes proton conductance inhibited by nucleotides and requires activation by ROS; may attenuate ROS production and protect against oxidative damage; possibly catalyzes fatty acid transport
PMID: 18074632
5
UCP3 variants (p.Val192Ile) are associated with severe early-onset obesity and metabolic disturbances including insulin resistance
PMID: 40281302
6
UCP3 gene variants contribute to obesity risk in Pakistani populations, with novel variants identified in regulatory regions and protein-coding sequences
PMID: 41006481
Disease Associationsⓘ21
morbid obesityOpen Targets
0.33Weak
obesityOpen Targets
0.24Weak
inherited obesityOpen Targets
0.17Weak
MODYOpen Targets
0.08Suggestive
neoplasmOpen Targets
0.07Suggestive
type 1 diabetes mellitusOpen Targets
0.07Suggestive
type 2 diabetes mellitusOpen Targets
0.07Suggestive
myocardial infarctionOpen Targets
0.06Suggestive
maturity-onset diabetes of the young type 4Open Targets
0.05Suggestive
diabetes mellitus, transient neonatal, 2Open Targets
0.05Suggestive
FRAXF syndromeOpen Targets
0.05Suggestive
maturity-onset diabetes of the young type 2Open Targets
0.05Suggestive
maturity-onset diabetes of the young type 6Open Targets
0.05Suggestive
hypertensionOpen Targets
0.05Suggestive
ischemiaOpen Targets
0.05Suggestive
maturity-onset diabetes of the young type 3Open Targets
0.05Suggestive
prediabetes syndromeOpen Targets
0.05Suggestive
Insulin resistanceOpen Targets
0.05Suggestive
maturity-onset diabetes of the young type 10Open Targets
0.05Suggestive
mosaic variegated aneuploidy syndrome 4Open Targets
0.05Suggestive
ObesityUniProt
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PPARGProtein interaction100%ADRB3Protein interaction92%CPT1AProtein interaction92%CPT1CProtein interaction91%PPARGC1AProtein interaction84%PPARAProtein interaction74%
Tissue Expression6 tissues
Lung
100%
Brain
59%
Ovary
59%
Liver
56%
Bone Marrow
49%
Heart
48%
Gene Interaction Network
Click a node to explore
UCP3PPARGADRB3CPT1ACPT1CPPARGC1APPARA
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt P55916
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.20LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.85 [0.62–1.20]
RankingsWhere UCP3 stands among ~20K protein-coding genes
  • #2,191of 20,598
    Most Researched195 · top quartile
  • #12,601of 17,882
    Most Constrained (LOEUF)1.20
Genes detectedUCP3
Sources retrieved31 papers
Response time—
📄 Sources
31▼
1
Muscle-UCP3 in the regulation of energy metabolism.
PMID: 38499130
Mitochondrion · 2024
1.00
2
Nucleosome assembly protein-like 1 degradation-dependent novel cardioprotection mechanism of Wnt2 against ischemia‒reperfusion injury.
PMID: 41397965
Signal Transduct Target Ther · 2025
0.90
3
GH directly stimulates UCP3 expression.
PMID: 29398371
Growth Horm IGF Res · 2018
0.84
4
Mitochondrial proton leaks and uncoupling proteins.
PMID: 33798544
Biochim Biophys Acta Bioenerg · 2021
0.80
5
Novel uncoupling proteins.
PMID: 18074632
Novartis Found Symp · 2007
0.80