The UMD-FBN1 mutations database
Record ID: 2224

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS3-2A>G (c.347-2A>G)HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ATAIlespl-2Spl.A->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
EGF-like #02 

Mutation impact


At the mRNA levelOn restriction map
Insertion of 1bp (intron 3)New restriction site(s): none
Lost restriction site(s): none

Impact on splicing
Splice site type Wild type sequence CV Mutant type sequence CV Variation (%)
Acceptor
ttttttttcaagTA
83.9 _
ttttttttcaggTA
54.9 _ *
-34.5 %

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
FRA01BOU F0480 I0517ProbandFemalefamilialFRANCE

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom

Reference


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