The UMD-FBN1 mutations database
Record ID: 199

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1511G>Tp.Cys504PheHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGTCysTTTPheG->TTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #03 Disulfide bonds 494-504 (C3)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)
0.670.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
GER03HAN F0009 I01ProbandFemalefamilial? (32 years old)GERMANY

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Mitral valve prolapse
O-Ectopia lentis

Reference


Reference IDPubMed IDReference
6610425041
Abd El-Aleem A, Karck M, Haverich A, Schmidtke J, Arslan-Kirchner M. "Identification of 9 novel FBN1 mutations in German patients with Marfan syndrome". Hum Mutation 1999 Aug 19;14(2):181.