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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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PPARGC1B
PPARG coactivator 1 beta
Chromosome 5 · 5q32
NCBI Gene: 133522Ensembl: ENSG00000155846.18HGNC: HGNC:30022UniProt: B7ZM40
121PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nucleusnucleoplasmmediator complexestrogen receptor signaling pathwayneurodegenerative diseasecutaneous melanomabasal cell carcinomaskin cancer
✦AI Summary

PPARGC1B (PGC-1β) is a transcriptional coactivator that serves as a master regulator of mitochondrial biogenesis and energy metabolism. It activates transcription factors including estrogen receptor alpha, nuclear respiratory factor 1 (NRF1), and glucocorticoid receptor [UniProt], and works downstream of nuclear receptors like RORγ and ERRα to coordinate oxidative phosphorylation (OXPHOS) programs 1. PPARGC1B overexpression increases basal oxygen consumption and mitochondrial number while promoting fat oxidation and energy expenditure regulation [UniProt]. At the molecular level, PPARGC1B functions through protein-binding interactions and mediator complex recruitment to positively regulate RNA polymerase II-dependent transcription and mitochondrial gene expression [GO annotations]. In disease contexts, PPARGC1B dysregulation contributes to cancer progression: RORγ-induced PGC-1β expression drives OXPHOS hyperactivation in osteosarcoma, supporting tumor growth 1, while PGC-1β degradation mediates sorafenib resistance in hepatocellular carcinoma by suppressing ROS and maintaining mitochondrial integrity 2. Conversely, IL-6-mediated suppression of PGC-1β expression impairs mitochondrial function during chr5 inflammation 3. Genetic variation at rs10071329 modulates PPARGC1B expression levels, with the G/G genotype enhancing expression and improving brown adipocyte mitochondrial respiration and lipolysis 4, suggesting clinical potential for precision medicine approaches targeting PPARGC1B in metabolic disorders.

Sources cited
1
RORγ induces PGC-1β expression and physically interacts with it to activate the OXPHOS program by upregulating respiratory chain component genes in osteosarcoma
PMID: 38692271
2
Upregulated UBQLN1 induces PGC-1β degradation to attenuate mitochondrial biogenesis and ROS production in sorafenib-resistant hepatocellular carcinoma cells
PMID: 34001851
3
rs10071329 G/G genotype enhances PPARGC1B expression with consequent improved mitochondrial function and norepinephrine response in brown adipocytes
PMID: 38190589
4
IL-6 suppresses mitochondrial function in settings where PGC-1β expression is low through the JAK1/STAT1/3-HIF1α/ERRα axis
PMID: 40056415
⚠Limited data available — This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.55Moderate
cutaneous melanomaOpen Targets
0.39Weak
basal cell carcinomaOpen Targets
0.36Weak
skin cancerOpen Targets
0.35Weak
trauma complicationOpen Targets
0.34Weak
response to TNF antagonistOpen Targets
0.32Weak
skin neoplasmOpen Targets
0.32Weak
frecklesOpen Targets
0.32Weak
seborrheic keratosisOpen Targets
0.31Weak
actinic keratosisOpen Targets
0.31Weak
polyarteritis nodosaOpen Targets
0.27Weak
cataractOpen Targets
0.26Weak
skin agingOpen Targets
0.26Weak
liver diseaseOpen Targets
0.26Weak
skin diseaseOpen Targets
0.25Weak
sunburnOpen Targets
0.25Weak
keratinocyte carcinomaOpen Targets
0.25Weak
suntanOpen Targets
0.25Weak
squamous cell carcinomaOpen Targets
0.24Weak
systemic lupus erythematosusOpen Targets
0.21Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
MED21Protein interaction100%MED1Protein interaction100%MED6Protein interaction100%MED11Protein interaction100%MED14Protein interaction100%MED26Protein interaction100%
Tissue Expression6 tissues
Heart
100%
Lung
48%
Liver
25%
Bone Marrow
24%
Brain
22%
Ovary
13%
Gene Interaction Network
Click a node to explore
PPARGC1BMED21MED1MED6MED11MED14MED26
PROTEIN STRUCTURE
Preparing viewer…
PDB3SP6 · 2.21 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.44Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.33 [0.25–0.44]
RankingsWhere PPARGC1B stands among ~20K protein-coding genes
  • #3,893of 20,598
    Most Researched121 · top quartile
  • #2,446of 17,882
    Most Constrained (LOEUF)0.44 · top quartile
Genes detectedPPARGC1B
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Tumor mitochondrial oxidative phosphorylation stimulated by the nuclear receptor RORγ represents an effective therapeutic opportunity in osteosarcoma.
PMID: 38692271
Cell Rep Med · 2024
1.00
2
UBQLN1 mediates sorafenib resistance through regulating mitochondrial biogenesis and ROS homeostasis by targeting PGC1β in hepatocellular carcinoma.
PMID: 34001851
Signal Transduct Target Ther · 2021
0.90
3
Human Genetic Variation at rs10071329 Correlates With Adiposity-Related Traits, Modulates PPARGC1B Expression, and Alters Brown Adipocyte Function.
PMID: 38190589
Diabetes · 2024
0.80
4
The pro-inflammatory cytokine IL6 suppresses mitochondrial function via the gp130-JAK1/STAT1/3-HIF1α/ERRα axis.
PMID: 40056415
Cell Rep · 2025
0.70
5
Inhibition of the PI3K-AKT-MTORC1 axis reduces the burden of the m.3243A>G mtDNA mutation by promoting mitophagy and improving mitochondrial function.
PMID: 39667405
Autophagy · 2025
0.60