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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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PPP1R15B
protein phosphatase 1 regulatory subunit 15B
Chromosome 1 · 1q32.1
NCBI Gene: 84919Ensembl: ENSG00000158615.10HGNC: HGNC:14951UniProt: A0A8I5KSH1
53PubMed Papers
21Diseases
0Drugs
0Pathogenic Variants
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein phosphatase type 1 complexprotein phosphatase 1 bindingprotein phosphatase regulator activitymolecular adaptor activitymicrocephaly, short stature, and impaired glucose metabolism 2primary microcephaly-mild intellectual disability-young-onset diabetes syndromeneurodegenerative diseaseParkinson disease
✦AI Summary

PPP1R15B (protein phosphatase 1 regulatory subunit 15B) is a regulatory subunit that functions as a critical regulator of the integrated stress response (ISR) by controlling eIF2α phosphorylation levels. In unstressed cells, PPP1R15B maintains low eIF2α phosphorylation by promoting its dephosphorylation through the PP1 phosphatase complex 1. Mechanistically, PPP1R15B contains a substrate-recruitment module with three helical elements that captures the trimeric eIF2 complex, positioning it for PP1-mediated dephosphorylation despite eIF2B inhibition 2. This function is essential for maintaining normal protein synthesis and cellular homeostasis. PPP1R15B dysfunction causes severe disease. A homozygous R658C mutation, located within the PP1-binding site, impairs PPP1R15B-PP1 complex stability, resulting in chr1 ISR activation and causing microcephaly, short stature, intellectual disability, and developmental delay 34. The cognitive deficits directly result from sustained ISR activation impairing synaptic function and memory 4. PPP1R15B is also implicated in cancer drug resistance. In glioblastoma, multiple myeloma, and hepatocellular carcinoma, PPP1R15B is upregulated through super-enhancer acquisition and drives drug-tolerant persister cell programs by maintaining protein homeostasis and redox buffering 567. Pharmacological PPP1R15B inhibition potentiates chemotherapy and targeted therapy responses, suggesting therapeutic promise in overcoming resistance.

Sources cited
1
PPP1R15B enables dephosphorylation of P-eIF2 by rescuing eIF2B from inhibition and terminating the integrated stress response
PMID: 41231936
2
PPP1R15B recruits trimeric eIF2 substrate via a disordered substrate-recruitment module; N423D variant impairs substrate binding in microcephaly syndrome
PMID: 38159565
3
Homozygous R658C mutation in PPP1R15B causes microcephaly, short stature, intellectual disability through impaired PP1C binding and elevated eIF2α phosphorylation
PMID: 26307080
4
PPP1R15B R658C variant destabilizes PP1 complex, causes chronic ISR activation, impairs memory and synaptic function; DP71L viral ortholog reverses cognitive deficits
PMID: 40568171
5
PPP1R15B super-enhancer activation drives drug-tolerant persister cells in glioblastoma; RAPHIN-1 inhibition enhances TMZ sensitivity
PMID: 40639044
6
PPP1R15B super-enhancer acquisition in multiple myeloma regulates protein homeostasis; Raphin1 inhibition activates eIF2α-ATF4-CHOP apoptosis and potentiates bortezomib
PMID: 39122682
7
PPP1R15B-ATF4-GPX4 axis sustains lenvatinib-tolerant persister cells in HCC through redox buffering; PPP1R15B inhibition restores ferroptosis sensitivity
PMID: 41513008
Disease Associationsⓘ21
microcephaly, short stature, and impaired glucose metabolism 2Open Targets
0.63Moderate
primary microcephaly-mild intellectual disability-young-onset diabetes syndromeOpen Targets
0.50Moderate
neurodegenerative diseaseOpen Targets
0.46Moderate
Parkinson diseaseOpen Targets
0.46Moderate
Alzheimer diseaseOpen Targets
0.46Moderate
lysosomal storage diseaseOpen Targets
0.46Moderate
multiple sclerosisOpen Targets
0.46Moderate
ovarian neoplasmOpen Targets
0.27Weak
genetic disorderOpen Targets
0.19Weak
Miyoshi myopathyOpen Targets
0.07Suggestive
multiple myelomaOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.07Suggestive
Hereditary persistence of fetal hemoglobin - beta-thalassemiaOpen Targets
0.06Suggestive
Blackfan-Diamond anemiaOpen Targets
0.06Suggestive
hemoglobin D diseaseOpen Targets
0.05Suggestive
hereditary elliptocytosisOpen Targets
0.05Suggestive
Congenital dyserythropoietic anemia type IOpen Targets
0.05Suggestive
X-linked sideroblastic anemia 1Open Targets
0.05Suggestive
Beta-thalassemia - X-linked thrombocytopeniaOpen Targets
0.05Suggestive
beta-thalassemia-X-linked thrombocytopenia syndromeOpen Targets
0.05Suggestive
Microcephaly, short stature, and impaired glucose metabolism 2UniProt
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
EIF2S1Protein interaction100%PPP1CAProtein interaction100%EIF1Protein interaction100%ACTA1Protein interaction90%PPP1CBProtein interaction89%PPP1CCProtein interaction89%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
23%
Lung
22%
Brain
17%
Ovary
14%
Heart
13%
Gene Interaction Network
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PPP1R15BEIF2S1PPP1CAEIF1ACTA1PPP1CBPPP1CC
PROTEIN STRUCTURE
Preparing viewer…
PDB4V0W · 1.55 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.73LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.55 [0.41–0.73]
RankingsWhere PPP1R15B stands among ~20K protein-coding genes
  • #8,479of 20,598
    Most Researched53
  • #5,745of 17,882
    Most Constrained (LOEUF)0.73
Genes detectedPPP1R15B
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Termination of the integrated stress response.
PMID: 41231936
Science · 2026
1.00
2
Super-enhancer PPP1R15B/EIF2A axis characterizes aggressive drug-tolerant persister cells in Glioblastoma.
PMID: 40639044
Biomed Pharmacother · 2025
0.90
3
Harnessing the Evolution of Proteostasis Networks to Reverse Cognitive Dysfunction.
PMID: 40568171
bioRxiv · 2025
0.80
4
Super enhancer acquisition drives expression of oncogenic PPP1R15B that regulates protein homeostasis in multiple myeloma.
PMID: 39122682
Nat Commun · 2024
0.70
5
Recruitment of trimeric eIF2 by phosphatase non-catalytic subunit PPP1R15B.
PMID: 38159565
Mol Cell · 2024
0.60