NRAS

Chr 1AD

NRAS proto-oncogene, GTPase

Also known as: ALPS4, CMNS, N-ras, NCMS, NRAS1, NS6, RALD1

NRAS encodes a membrane-associated GTPase that shuttles between the Golgi apparatus and plasma membrane and functions in cellular signaling pathways. Germline mutations cause Noonan syndrome 6 with autosomal dominant inheritance, while somatic mutations result in various conditions including epidermal nevi, melanocytic nevus syndromes, and RAS-associated autoimmune lymphoproliferative syndrome. The gene is moderately constrained against loss-of-function variants (LOEUF 0.612), and both germline and somatic variants can affect multiple organ systems including cardiac, cutaneous, and hematologic.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
GOFmechanismADLOEUF 0.618 OMIM phenotypes
VCEP Guidelines: RASopathyPilot
View SpecificationsClinGen Panel
Clinical SummaryNRAS
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Gene-Disease Validity (ClinGen)
Costello syndrome · ADLimited

Limited evidence — not for standalone diagnostic reporting

4 total gene-disease associations curated

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.19) despite low pLI — interpret in context.
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Clinical Trials
12 active or recruiting trials — potential therapeutic options may be available
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GeneReview available — NRAS
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.61LOEUF
pLI 0.490
Z-score 2.39
OE 0.19 (0.080.61)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.71Z-score
OE missense 0.53 (0.420.66)
55 obs / 104.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.19 (0.080.61)
00.351.4
Missense OE0.53 (0.420.66)
00.61.4
Synonymous OE1.00
01.21.6
LoF obs/exp: 2 / 10.3Missense obs/exp: 55 / 104.2Syn Z: 0.01
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveNRAS-related Noonan syndromeGOFAD
DN
0.75top 25%
GOF
0.76top 25%
LOF
0.3745th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function, dominant-negative and loss-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median · 1 literature citation
DNprediction above median · 1 literature citation
LOF1 literature citation

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Literature Evidence

DNStructure of the dominant negative S17N mutant of RasPMID:20131908
GOFActivating germline mutations of KRAS and HRAS cause severe developmental abnormalities leading to Noonan, cardio-facial-cutaneous, and Costello syndrome, but activating germline mutations of NRAS have not been reported.PMID:17517660
LOF1p13.2 deletion displays clinical features overlapping Noonan syndrome, likely related to NRAS gene haploinsufficiency.PMID:27561113

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

NRAS · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

NaeviNeurodevelopmental DisorderCongenital Nevus

CongenItal Naevus Cohort for Longitudinal Evaluation

RECRUITING
NCT06828822Phase NANantes University HospitalStarted 2025-07-23
Neurodevelopmental assessmentMeeting with the parentsPatient quality of life assessment
OncologyMEK MutationRAF Gene Mutation

A Study to Investigate the Safety and Efficacy of NST-628 Oral Tablets in Subjects With Solid Tumors

RECRUITING
NCT06326411Phase PHASE1Nested Therapeutics, IncStarted 2024-04-09
NST-628
Metastatic Colorectal Cancer (mCRC)Colorectal Neoplasms

Efficacy And Safety Of Hydroxychloroquine Combined With Methotrexate, Capecitabine And Bevacizumab Vs. Regorafenib In Participants With Refractory Metastatic Colorectal Cancer With Mutations In RAS Genes

RECRUITING
NCT06949982Phase PHASE2Sergey Orlov, MDStarted 2025-03-17
experimental group x-MAPRegorafenib (BAY 73-4506)
Non-small Cell Lung CancerHistiocytic NeoplasmHistiocytosis

Phase 1/2 Trial of S241656 in Selected RAS/MAPK Mutation- Positive Malignancies

RECRUITING
NCT05786924Phase PHASE1, PHASE2Institut de Recherches Internationales ServierStarted 2023-04-18
S241656FOLFOX6/FOLFOX7FOLFIRI
Colorectal Cancer

An Exploratory Study of Treatment Sensitivity and Prognostic Factors in a Efficacy and Safety Study of mFOLFOX6 + Bevacizumab Versus mFOLFOX6 + Panitumumab Therapy in Patients With Chemotherapy-naïve Unresectable Advanced or Recurrent Colorectal Cancer

ACTIVE NOT RECRUITING
NCT02394834TakedaStarted 2015-05-29
oxaliplatin (OXA), levofolinate calcium (l-LV), 5-FU, panitumumaboxaliplatin (OXA), levofolinate calcium (l-LV), 5-FU, bevacizumab
RASopathy

Study of the Thyroid Function and Echostructural Morphology in Patients Affected With Rasopathies (ECORAS2023)

RECRUITING
NCT06489067University of Bari Aldo MoroStarted 2024-01-15
Colorectal Cancer

Tracking Mutations in Cell Free Tumour DNA to Predict Relapse in Early Colorectal Cancer

RECRUITING
NCT04050345Royal Marsden NHS Foundation TrustStarted 2016-12-05
Non Small Cell Lung CancerEGFR Gene MutationALK Gene Mutation

Treatment Strategies and Survival Outcome for Non-small Cell Lung Cancer With Oncogenic Mutation

RECRUITING
NCT04322890Phase PHASE2Hunan Province Tumor HospitalStarted 2020-04-16
OsimertinibAlectinib 150 MGCrizotinib 250 MG
CancerMalignancies MultipleMalignant Solid Tumor

CAnadian CAncers With Rare Molecular Alterations (CARMA) - Basket Real-world Observational Study (BROS)

RECRUITING
NCT04151342University Health Network, TorontoStarted 2020-01-17
Cancer treatment with tyrosine kinase inhibitors (TKIs) or other molecularly targeted therapeutic agents.Patient-reported outcomes (PROs)
Leukemia, Myeloid, AcuteRefractory AMLRelapsed Adult AML

Clinical Trial to Evaluate the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Tuspetinib (HM43239) in Patients With Relapsed or Refractory Acute Myeloid Leukemia

RECRUITING
NCT03850574Phase PHASE1, PHASE2Aptose Biosciences Inc.Started 2019-03-11
TuspetinibVenetoclax Oral TabletAzacitidine for Intravenous Infusion
Rectal Neoplasms

MRI Simulation-guided Boost in Short-course Preoperative Radiotherapy for Unresectable Rectal Cancer

RECRUITING
NCT03714490Phase PHASE2Cancer Institute and Hospital, Chinese Academy of Medical SciencesStarted 2018-10-23
SCPRTCRTCAPOX
Melanoma

Effects of Anti-PD1 Adjuvant Checkpoint Blockade Immunotherapy on Atypical/Dysplastic Nevi

RECRUITING
NCT06599619John KirkwoodStarted 2025-02-20
Single agent, adjuvant anti-PD1 therapy
Clinical Literature
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Key Publications
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