vabase

NRAS Q61R

c.182A>GNM_002524.41-114713908-T-C
RAS-MAPK gain of function transition Cancer hotspot Absent from gnomAD ClinVar: Pathogenic AlphaMissense: likely pathogenic KLAGLACCLA

Reports 3 in KLA · GLA · CCLA

ISSVA entityAllele fractionSources
KLA Barclay 2019: 1-28%; Li 2023: 0.54-5.1%; Brown 2026: 4.69% (pleural-fluid cfDNA)
GLA 30%
CCLA not reported

Bars show the reported allele-fraction range on a 0 to 50 percent axis.

Position in NRAS 2 catalogued variants in this gene

1189 aaQ61KQ61R

Pathway and targeted therapy

Variant in the RAS-MAPK pathway. Repurposed drugs already used in the clinic for this pathway: trametinib and other MEK inhibitors.

Open and recent trials (3 in vascular / lymphatic anomalies for trametinib)

Primary sources 5

  1. Resolution of Refractory Chylous Effusions With Targeted MEK Inhibition in NRAS Q61R-Driven Kaposiform Lymphangiomatosis: A Case Report.
    Brown G, McNab S, Keogh S, Massie J, Eastaugh L, Pathmanathan L, Bertinetti M, Sullivan M, Orme L, Cloney T, Brown NJ, Tan TY, Quin R, Pertile M, Moon A, Bekhit E, Penington T. · Case Rep Pediatr · 2026
  2. NRAS mutation in a central conducting lymphatic anomaly and PPFIBP1::ROS1 fusion in a Gorham-stout disease patient.
    Yang G, Ren H, Wang J, Chen W, Li X, Chen S, Chen H, Zhao L, Fan W, Xiao S. · Hum Mol Genet · 2026
  3. Genomic profiling informs diagnoses and treatment in vascular anomalies.
    Li D, Sheppard SE, March ME, Battig MR, Surrey LF, Srinivasan AS, Matsuoka LS, Tian L, Wang F, Seiler C, Dayneka J, Borst AJ, Matos MC, Paulissen SM, Krishnamurthy G, Nriagu B, Sikder T, Casey M, Williams L, Rangu S, O'Connor N, Thomas A, Pinto E, Hou C, Nguyen K, Pellegrino da Silva R, Chehimi SN, Kao C, Biroc L, Britt AD, Queenan M, Reid JR, Napoli JA, Low DM, Vatsky S, Treat J, Smith CL, Cahill AM, Snyder KM, Adams DM, Dori Y, Hakonarson H. · Nat Med · 2023
  4. A somatic activating NRAS variant associated with kaposiform lymphangiomatosis.
    Barclay SF, Inman KW, Luks VL, McIntyre JB, Al-Ibraheemi A, Church AJ, Perez-Atayde AR, Mangray S, Jeng M, Kreimer SR, Walker L, Fishman SJ, Alomari AI, Chaudry G, Trenor Iii CC, Adams D, Kozakewich HPW, Kurek KC. · Genet Med · 2019
  5. Somatic NRAS mutation in patient with generalized lymphatic anomaly.
    Manevitz-Mendelson E, Leichner GS, Barel O, Davidi-Avrahami I, Ziv-Strasser L, Eyal E, Pessach I, Rimon U, Barzilai A, Hirshberg A, Chechekes K, Amariglio N, Rechavi G, Yaniv K, Greenberger S. · Angiogenesis · 2018

Mentioned in 1,690 publications indexed by Europe PMC.

Annotations refreshed 2026-10-09 from Europe PMC, ClinVar, ClinicalTrials.gov, gnomAD, and Genome Nexus. Coordinates handled per assembly (gnomAD GRCh38, Genome Nexus GRCh37).