The UMD-FBN1 mutations database
Record ID: 821

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.643C>Tp.Arg215XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGAArgTGAStopC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
Hybrid module#01 Yes, coding strandYes

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Dde I, Esp I
Lost restriction site(s): Eag I, Hae III

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD05LON F0029 I01ProbandNANAU.K.

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


SymptomAge
C-Minor cardiovascular involvement13

Reference


Reference IDPubMed IDReference
9617657824
Comeglio P, Johnson P, Arno G, Brice G, Evans A, Aragon-Martin J, da Silva FP, Kiotsekoglou A, Child A. "The importance of mutation detection in Marfan syndrome and Marfan-related disorders: report of 193 FBN1 mutations". Hum Mutat. 2007 Sep;28(9):928.