The UMD-FBN1 mutations database
Record ID: 2643

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS35-8G>A (c.4460-8G>A)HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GATAspspl-8Spl.G->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #22 Ca2+ binding

Mutation impact


At the mRNA levelOn restriction map
Insertion of 6bp (intron 35)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 (%)
Cryptic Acceptor?
CTCACTGTTCGGTTT
57 _
CTCACTGTTCAGTTT
86 _ *
33.7 %

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
FRA01BOU F0905 I0941ProbandFemalede novoFRANCE

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


Symptom

Reference


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