The UMD-FBN1 mutations database
Record ID: 839

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3420C>Ap.Cys1140XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGCCysTGAStopC->ATv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #13 Disulfide bonds 1140-1153 (C5)Yes, coding strandNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): BspW I

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD05LON F0047 I01ProbandNANAU.K.

Phenotypic groupDisease
NAClassical MFS

Clinical data


SymptomAge
C-Dilation or dissection of asc. aorta40

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.