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50 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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PPARGC1A
PPARG coactivator 1 alpha
Chromosome 4 · 4p15.2
NCBI Gene: 10891Ensembl: ENSG00000109819.11HGNC: HGNC:9237UniProt: Q9UBK2
807PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Hub GeneTranscription Factor
RESEARCH IMPACT
Highly StudiedTrending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nucleuspositive regulation of transcription by RNA polymerase IIubiquitin protein ligase bindingsequence-specific DNA bindingheart conduction diseaseLeft bundle branch blockplacenta praeviaconduction system disorder
✦AI Summary

PPARGC1A (PGC-1α) is a master transcriptional coactivator that coordinates cellular energy metabolism and mitochondrial function 1. As a key regulator of nuclear receptors including PPARG and thyroid hormone receptor, PGC-1α stimulates mitochondrial biogenesis and promotes adaptive thermogenesis in response to environmental stimuli like cold exposure 1. Beyond biogenesis, PGC-1α regulates mitochondrial quality control mechanisms including fission, fusion, and mitophagy, with implications for insulin sensitivity and age-related dysfunction 2. The protein orchestrates metabolic reprogramming by controlling genes involved in gluconeogenesis, glucose and fatty acid metabolism, and lipid homeostasis 3. PPARGC1α has isoform-specific functions: the canonical form regulates oxidative phosphorylation and endurance adaptations, while the PGC-1α4 isoform induced by resistance training promotes skeletal muscle hypertrophy through IGF1 induction and myostatin repression 4. Clinically, PPARGC1A dysfunction associates with metabolic disorders including obesity, Type 2 diabetes, and age-related macular degeneration characterized by lipid accumulation 13. Cancer cells exploit PGC-1α-mediated mitochondrial respiration to enhance metastatic potential 5, making PPARGC1A an emerging therapeutic target across metabolic and oncologic diseases.

Sources cited
1
PGC-1α regulates energy metabolism, is induced by cold exposure, stimulates mitochondrial biogenesis, and is involved in obesity, diabetes, and cardiomyopathy
PMID: 17108241
2
PGC-1α regulates mitochondrial quality control beyond biogenesis, including fission, fusion, and mitophagy, with effects on insulin sensitivity
PMID: 32516539
3
PGC-1α repression dysregulates lipid metabolism and causes lipid droplet accumulation in retinal pigment epithelium, relevant to age-related macular degeneration
PMID: 38824126
4
PGC-1α4 isoform induced by resistance training promotes skeletal muscle hypertrophy through IGF1 induction and myostatin repression
PMID: 23217713
5
PGC-1α enhances oxidative phosphorylation and mitochondrial biogenesis in invasive cancer cells and correlates with metastatic potential
PMID: 25241037
Disease Associationsⓘ20
heart conduction diseaseOpen Targets
0.43Moderate
Left bundle branch blockOpen Targets
0.42Moderate
placenta praeviaOpen Targets
0.40Weak
conduction system disorderOpen Targets
0.39Weak
smoking initiationOpen Targets
0.39Weak
amyotrophic lateral sclerosisOpen Targets
0.39Weak
bundle branch blockOpen Targets
0.38Weak
Complete right bundle branch blockOpen Targets
0.38Weak
neurodegenerative diseaseOpen Targets
0.36Weak
kidney failureOpen Targets
0.36Weak
severe acute respiratory syndromeOpen Targets
0.32Weak
COVID-19Open Targets
0.32Weak
atrioventricular blockOpen Targets
0.31Weak
schizophreniaOpen Targets
0.31Weak
renal carcinomaOpen Targets
0.31Weak
Abnormality of refractionOpen Targets
0.30Weak
idiopathic pulmonary arterial hypertensionOpen Targets
0.29Weak
Abnormality of the skeletal systemOpen Targets
0.28Weak
acquired thrombocytopeniaOpen Targets
0.28Weak
hyperpituitarismOpen Targets
0.28Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
BMAL1Protein interaction100%ESRRAProtein interaction100%FOXO3Protein interaction100%NRF1Protein interaction100%PPARGProtein interaction100%SREBF1Protein interaction100%
Tissue Expression6 tissues
Heart
100%
Liver
51%
Brain
12%
Lung
5%
Ovary
2%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
PPARGC1ABMAL1ESRRAFOXO3NRF1PPARGSREBF1
PROTEIN STRUCTURE
Preparing viewer…
PDB9F7W · 1.25 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.38Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.27 [0.20–0.38]
RankingsWhere PPARGC1A stands among ~20K protein-coding genes
  • #238of 20,598
    Most Researched807 · top 5%
  • #1,826of 17,882
    Most Constrained (LOEUF)0.38 · top quartile
Genes detectedPPARGC1A
Sources retrieved50 papers
Response time—
📄 Sources
50▼
1
PGC-1alpha: a key regulator of energy metabolism.
PMID: 17108241
Adv Physiol Educ · 2006
1.00
2
Sirt1 and the Mitochondria.
PMID: 26831453
Mol Cells · 2016
0.90
3
Effects of
PMID: 36466447
Front Public Health · 2022
0.82
4
CLU (clusterin) and PPARGC1A/PGC1α coordinately control mitophagy and mitochondrial biogenesis for oral cancer cell survival.
PMID: 38447939
Autophagy · 2024
0.80
5
A branched-chain amino acid metabolite drives vascular fatty acid transport and causes insulin resistance.
PMID: 26950361
Nat Med · 2016
0.80