SET

Chr 9

SET nuclear proto-oncogene

Also known as: 2PP2A, I2PP2A, IGAAD, IPP2A2, MRD58, PHAPII, TAF-I, TAF-IBETA

The protein encoded by this gene inhibits acetylation of nucleosomes, especially histone H4, by histone acetylases (HAT). This inhibition is most likely accomplished by masking histone lysines from being acetylated, and the consequence is to silence HAT-dependent transcription. The encoded protein is part of a complex localized to the endoplasmic reticulum but is found in the nucleus and inhibits apoptosis following attack by cytotoxic T lymphocytes. This protein can also enhance DNA replication of the adenovirus genome. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2011]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtIntellectual developmental disorder, autosomal dominant 58

Clinical highlights

Gene-disease validity (ClinGen)
intellectual disability · ADDefinitivesufficient evidence for diagnostic panels
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype) and the gene is intolerant of it in the population — truncating, frameshift and canonical splice variants carry more prior weight here than missense.Curated gene-level mechanism — a prior for triage, not a per-variant call.
12
Active trials
79909
Pubs (1 yr)
P/LP submissions
P/LP missense
0.18
LOEUF· LoF intol.
LOF
Mechanism· G2P
📖
GeneReview available — SET
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint?
0.18LOEUF
pLI 0.996
Z-score 3.74
OE 0.00 (0.000.18)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint?
2.66Z-score
OE missense 0.38 (0.300.47)
55 obs / 145.1 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios?
LoF OE?0.00 (0.000.18)
00.351.4
Missense OE?0.38 (0.300.47)
00.61.4
Synonymous OE?1.10
01.21.6
LoF obs/exp: 0 / 16.3Missense obs/exp: 55 / 145.1Syn Z: -0.60

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SET · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

Advanced Bladder CarcinomaAdvanced Genitourinary System CarcinomaMetastatic Bladder Carcinoma

Testing Olaparib in Patients With Advanced or Metastatic (Cancer That Has Spread) Bladder Cancer and Other Genitourinary Tumors With DNA-Repair Genetic Changes

ACTIVE NOT RECRUITING
NCT03375307Phase PHASE2National Cancer Institute (NCI)Started 2020-11-03
Biopsy ProcedureBiospecimen CollectionBone Marrow Biopsy
Glioblastoma, IDH-wildtype

Oxidative Phosphorylation Targeting In Malignant Glioma Using Metformin Plus Radiotherapy Temozolomide

RECRUITING
NCT04945148Phase PHASE2Hopital FochStarted 2024-05-10
MetforminRadiation IMRTTemozolomide
Prostate Cancer (Diagnosis)

miRNA Panel for PCa Diagnosis

RECRUITING
NCT07755592RenJi HospitalStarted 2026-04-13
Serum microRNA panel detection for prostate lesion differentiation
Congenital Myotonic DystrophyCDM

DMCRN-02-001: Assessing Pediatric Endpoints in DM1

RECRUITING
NCT05224778Virginia Commonwealth UniversityStarted 2022-08-24
Rare Diseases of Genetic OriginRare Forms of Common Diseases Suspected of Being Genetic in Origin

Identification of the Molecular and/or Pathophysiological Bases of Rare Diseases of Genetic Origin (or Rare Forms of Frequent Diseases Suspected of Being of Genetic Origin).

RECRUITING
NCT03287193Centre Hospitalier Universitaire DijonStarted 2017-03-13
Blood and/or tissue samplinghigh-throughput sequencing
B-cell Lymphoma

Anti CD19 Gene Therapy for B-cell Lymphoma

RECRUITING
NCT07211048Phase NAAnhui Provincial HospitalStarted 2026-03-20
Anti-CD19 gene injection
Hypertrophic Cardiomyopathy (HCM)

Role of Cardiovascular Imaging in the Early Diagnosis of Hypertrophic Cardiomyopathy in Women

ACTIVE NOT RECRUITING
NCT06882668IRCCS San RaffaeleStarted 2025-03-01
Anatomic Stage IV Breast Cancer AJCC v8Hormone Receptor Positive Breast CarcinomaMetastatic Breast Carcinoma

Using FDG-PET/CT to Assess Response of Bone-Dominant Metastatic Breast Cancer, FEATURE Study

ACTIVE NOT RECRUITING
NCT04316117Phase PHASE2ECOG-ACRIN Cancer Research GroupStarted 2020-09-15
Computed TomographyFludeoxyglucose F-18Positron Emission Tomography
Prediabetes

Training Induced Muscle-Adipose EV Communication

NOT YET RECRUITING
NCT07106450Phase NAYuan WenStarted 2026-09
Acute Resistance Exercise
Myeloproliferative Neoplasms

Registry of Patients With MPNs in Taiwan

ACTIVE NOT RECRUITING
NCT03618485Chang Gung Memorial HospitalStarted 2017-04-01
HematologyDiffused Large B Cell Lymphoma

A Multicenter Observational Study to Understand the Clinical Characteristics, Treatment Patterns and Access to Novel Therapies of Patients With Diffuse Large B-Cell Lymphoma in the MEA Region

RECRUITING
NCT07065344AstraZenecaStarted 2025-07-23
Pre-FrailtyAging

Exploratory Study on a Multi-nutrient Supplement and Exercise Program for Improving Health Markers Associated to Longevity in Pre-frail Adults

RECRUITING
NCT07454616Phase NANational University of SingaporeStarted 2025-09-22
Supervised exercise programa balanced micronutrient blend