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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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PPP2R3B
protein phosphatase 2 regulatory subunit B''beta
Chromosome X|Y · X;Y
NCBI Gene: 28227Ensembl: ENSG00000167393.19HGNC: HGNC:13417UniProt: Q9Y5P8
35PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingnucleoplasmprotein phosphatase type 2A complexprotein dephosphorylationmelanomafemur fractureneoplasmcongenital mesoblastic nephroma
✦AI Summary

PPP2R3B encodes PR70, a regulatory B subunit of protein phosphatase 2A (PP2A) that modulates substrate selectivity and subcellular localization of the catalytic enzyme 1. Located on the pseudoautosomal region of sex chrX|Y, PPP2R3B functions as a tumor suppressor in melanoma by negatively regulating DNA replication and cell cycle progression through stabilization of the CDC6-CDT1 interaction, which delays origin firing 1. Germline duplications of PPP2R3B predispose individuals to melanocytic nevi and melanoma by inducing a proliferative phenotype mediated through C21orf91, independent of the MITF pathway 2. The gene shows sex-specific expression patterns, with lower expression in males correlating with poor melanoma outcomes, supporting its role as an X-linked tumor suppressor 1. PPP2R3B is expressed during human fetal development at sites of chondrogenesis and in muscle tissues 3. Loss-of-function mutations in zebrafish cause progressive idiopathic scoliosis with reduced bone mineralization and mitochondrial abnormalities 3. The protein interacts with NOD2 in innate immune signaling, with interactions compromised by Crohn's disease-associated NOD2 mutations 4. PPP2R3B represents a therapeutically relevant PP2A subunit class that exhibits synthetic dosage lethality with PLK1 overexpression in multiple cancer types 5.

Sources cited
1
PPP2R3B encodes PR70 regulatory subunit of PP2A and functions as X-linked tumor suppressor in melanoma
PMID: 27974665
2
Germline duplications predispose to melanoma through C21orf91-mediated proliferative phenotype
PMID: 34145395
3
Loss-of-function causes idiopathic scoliosis in zebrafish and expression in human fetal development
PMID: 37100808
4
Protein interacts with NOD2 in immune signaling
PMID: 27812135
5
Represents therapeutically relevant PP2A subunit with synthetic lethality to PLK1 overexpression
PMID: 27557495
Disease Associationsⓘ20
melanomaOpen Targets
0.05Suggestive
femur fractureOpen Targets
0.02Suggestive
neoplasmOpen Targets
0.01Suggestive
congenital mesoblastic nephromaOpen Targets
0.01Suggestive
idiopathic scoliosisOpen Targets
0.01Suggestive
juvenile dermatomyositisOpen Targets
0.01Suggestive
large congenital melanocytic nevusOpen Targets
0.01Suggestive
KyphoscoliosisOpen Targets
0.01Suggestive
breast cancerOpen Targets
0.01Suggestive
cancerOpen Targets
0.00Suggestive
spinocerebellar ataxia type 12Open Targets
0.00Suggestive
Leri-Weill dyschondrosteosisOpen Targets
0.00Suggestive
scoliosisOpen Targets
0.00Suggestive
atopic eczemaOpen Targets
0.00Suggestive
nevusOpen Targets
0.00Suggestive
osteogenesis imperfectaOpen Targets
0.00Suggestive
prostate cancerOpen Targets
0.00Suggestive
gastric cancerOpen Targets
0.00Suggestive
glioblastomaOpen Targets
0.00Suggestive
metastatic melanomaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PPP2R1BProtein interaction98%CDC6Protein interaction95%PPP3CAProtein interaction95%PPP3CBProtein interaction95%PPP3R1Protein interaction95%PPP4R2Protein interaction95%
Tissue Expression6 tissues
Heart
100%
Ovary
29%
Lung
18%
Liver
17%
Bone Marrow
16%
Brain
7%
Gene Interaction Network
Click a node to explore
PPP2R3BPPP2R1BCDC6PPP3CAPPP3CBPPP3R1PPP4R2
PROTEIN STRUCTURE
Preparing viewer…
PDB4MEW · 1.99 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.14LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.93 [0.77–1.14]
RankingsWhere PPP2R3B stands among ~20K protein-coding genes
  • #11,043of 20,598
    Most Researched35
  • #11,857of 17,882
    Most Constrained (LOEUF)1.14
Genes detectedPPP2R3B
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Inherited duplications of PPP2R3B predispose to nevi and melanoma via a C21orf91-driven proliferative phenotype.
PMID: 34145395
Genet Med · 2021
1.00
2
A CRISPR/Cas9-generated mutation in the zebrafish orthologue of PPP2R3B causes idiopathic scoliosis.
PMID: 37100808
Sci Rep · 2023
0.90
3
The protein phosphatase 2A regulatory subunit PR70 is a gonosomal melanoma tumor suppressor gene.
PMID: 27974665
Sci Transl Med · 2016
0.80
4
Cytoprotective Effects of Human Platelet Lysate during the Xeno-Free Culture of Human Donor Corneas.
PMID: 36769200
Int J Mol Sci · 2023
0.70
5
Responses of gonadal transcriptome and physiological analysis following exposure to 17α-ethynylestradiol in adult rare minnow Gobiocypris rarus.
PMID: 28349872
Ecotoxicol Environ Saf · 2017
0.60