The UMD-FBN1 mutations database
Record ID: 1725

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1511G>Ap.Cys504TyrHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGTCysTATTyrG->ATs

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
UKD07WIL F0009 I0001ProbandMalefamilial? (45 years old)U.K.

Phenotypic groupDisease
NADominant ectopia lentis

Clinical data


SymptomAge
O-Ectopia lentis45

Reference


Reference IDPubMed IDReference
15717627385
Howarth R, Yearwood C, Harvey JF. "Application of dHPLC for mutation detection of the fibrillin-1 gene for the diagnosis of Marfan syndrome in a National Health Service Laboratory". Genet Test. 2007 Summer;11(2):146-52.