KEGG   VARIANT: 64121v1
Entry
64121v1                      Variant                               
Name
RRAGC mutation
Type
Gain of function
Gene
RRAGC  Ras related GTP binding C [KO:K16186]
Organism
hsa_var Human gene variants (Homo sapiens)
Variation
mutations
OmimVar: 608267
Network
nt06522  mTOR signaling
Disease
H02697  Long-Olsen-Distelmaier syndrome
Reference
  Authors
Long PA, Zimmermann MT, Kim M, Evans JM, Xu X, Olson TM
  Title
De novo RRAGC mutation activates mTORC1 signaling in syndromic fetal dilated cardiomyopathy.
  Journal
Hum Genet 135:909-917 (2016)
DOI:10.1007/s00439-016-1685-3
Reference
  Authors
Reijnders MRF, Seibt A, Brugger M, Lamers IJC, Ott T, Klaas O, Horvath J, Rose AMS, Craghill IM, Brunet T, Graf E, Mayerhanser K, Hellebrekers D, Pauck D, Neuen-Jacob E, Rodenburg RJT, Wieczorek D, Klee D, Mayatepek E, Driessen G, Bindermann R, Averdunk L, Lohmeier K, Sinnema M, Stegmann APA, Roepman R, Poulter JA, Distelmaier F
  Title
De novo missense variants in RRAGC lead to a fatal mTORopathy of early childhood.
  Journal
Genet Med 25:100838 (2023)
DOI:10.1016/j.gim.2023.100838
LinkDB

DBGET integrated database retrieval system