The UMD-FBN1 mutations database
Record ID: 1723

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1354delTp.Tyr452ThrfsX127HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TACTyrdel1aFs.Stop at 578Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
EGF-like #04 

Mutation impact


At the mRNA levelOn restriction map
Deletion in a stretch of nucleotidesNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD07WIL F0007 I0001ProbandFemaleNA? (45 years old)U.K.

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


SymptomSeverityAge
C-Mitral regurgitationsurgery45
O-Ectopia lentis45
S-Incomplete description45
SI-Incomplete description45

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.