NONO

Chr XX-linked

non-POU domain containing octamer binding

Also known as: MRXS34, NMT55, NRB54, P54, P54NRB, PPP1R114

This gene encodes an RNA-binding protein which plays various roles in the nucleus, including transcriptional regulation and RNA splicing. A rearrangement between this gene and the transcription factor E3 gene has been observed in papillary renal cell carcinoma. Alternatively spliced transcript variants have been described. Pseudogenes exist on Chromosomes 2 and 16. [provided by RefSeq, Feb 2009]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Intellectual developmental disorder, X-linked syndromic 34MIM #300967
X-linked

Clinical highlights

Gene-disease validity (ClinGen)
X-linked syndromic intellectual disability · XLDefinitivesufficient 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
107
Pubs (1 yr)
P/LP submissions
P/LP missense
0.24
LOEUF· LoF intol.
LOF
Mechanism· G2P
📖
GeneReview available — NONO
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint?
0.24LOEUF
pLI 0.993
Z-score 3.87
OE 0.05 (0.020.24)
Highly constrained

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

Missense Constraint?
3.59Z-score
OE missense 0.27 (0.220.34)
52 obs / 191.9 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios?
LoF OE?0.05 (0.020.24)
00.351.4
Missense OE?0.27 (0.220.34)
00.61.4
Synonymous OE?0.95
01.21.6
LoF obs/exp: 1 / 19.4Missense obs/exp: 52 / 191.9Syn Z: 0.32

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NONO · 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

Glioblastoma, IDH-wildtype

Oxidative Phosphorylation Targeting In Malignant Glioma Using Metformin Plus Radiotherapy Temozolomide

RECRUITING
NCT04945148Phase PHASE2Hopital FochStarted 2024-05-10
MetforminRadiation IMRTTemozolomide
Stage III Periodontitis

Active Oxygen Releasing Gel With Photodynamic Therapy in Treating Stage III Periodontitis

RECRUITING
NCT07368699Phase NAAlexandria UniversityStarted 2025-01-26
Scaling and root planing, topical active oxygen-releasing gel, and photodynamic therapy using diode laserScaling and root planing and topical application of active oxygen-releasing gelScaling and root planing
HealthyOverweight (BMI > 25)Overweight , Obesity

Effect of Probiotics on Appetite Regulation in Adults With Overweight/Obesity

RECRUITING
NCT07715955Phase NAMaastricht University Medical CenterStarted 2026-03-18
Probiotic supplementationPlacebo
Prostate Cancer (Diagnosis)

miRNA Panel for PCa Diagnosis

RECRUITING
NCT07755592RenJi HospitalStarted 2026-04-13
Serum microRNA panel detection for prostate lesion differentiation
Heart Failure

Registry of Patients with Suspected Amyloidosis in Heart Failure

ACTIVE NOT RECRUITING
NCT05176548Fundación Pública Andaluza para la gestión de la Investigación en SevillaStarted 2022-01-15
Bone-cardiac scintigraphy
ANCA Associated Vasculitis (AAV)

The Safety, Efficacy, and Cellular Metabolic Kinetics of CT1192 in Treating Patients With Anti Neutrophil Cytoplasmic Antibody Associated Vasculitis

NOT YET RECRUITING
NCT07033299Phase PHASE1Union Hospital, Tongji Medical College, Huazhong University of Science and TechnologyStarted 2025-07-01
CAR-T Therapy
Congenital Myotonic DystrophyCDM

DMCRN-02-001: Assessing Pediatric Endpoints in DM1

RECRUITING
NCT05224778Virginia Commonwealth UniversityStarted 2022-08-24
ObesityPreDiabetes

The Effect of Time-Restricted Eating in Cardiometabolic Health

RECRUITING
NCT05866406Phase NACambridge University Hospitals NHS Foundation TrustStarted 2023-11-01
Time restricted eatingExtended eating windowHealthy diet
Bleeding Disorder

Early Genomic Testing for Inherited Bleeding Disorders

RECRUITING
NCT06736158Phase EARLY_PHASE1Queen's UniversityStarted 2025-05-31
Genetic testing for inherited bleeding disorders
Type1 Diabetes Mellitus

EXtremely Early-onset Type 1 Diabetes EXtremely Early-onset Type 1 Diabetes (A Musketeers' Memorandum Study)

RECRUITING
NCT03369821University of ExeterStarted 2017-09-19
Beta Cell Loss and Immune FunctionImmune Function with RNAseq
SARS CoV 2 InfectionSARS CoV 2 Vaccination

COVID-19 Transmission and Morbidity in Malawi

RECRUITING
NCT05973084Boston UniversityStarted 2023-01-17
CMTCMT (Charcot Marie Tooth Disease)CMT - Charcot-Marie-Tooth Disease

A Multi-omic Approach to the Identification of Novel Biomarkers in Early Charcot-Marie-Tooth 1A Disease (CMT1A)

RECRUITING
NCT07476365University Medical Center GoettingenStarted 2025-03-24