The UMD-FBN1 mutations database
Record ID: 2589

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3349T>Cp.Cys1117ArgHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGTCysCGTArgT->CTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #13 Disulfide bonds 1117-1129 (C1)NoNo

Mutation impact


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

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.830.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
FRA01BOU F0846 I0882ProbandFemalede novoFRANCE

Phenotypic groupDisease
NANeonatal MFS

Clinical data


Symptom

Reference


Reference IDPubMed IDReference
207-
Boileau C. (personnal communication 2013)