The UMD-FBN1 mutations database
Record ID: 1788

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS11+5G>A (c.1468+5G>A)HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GATAspspl+5Spl.G->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #03 Ca2+ binding

Mutation impact


At the mRNA levelOn restriction map
Skipping of exon 11, frameshiftNew restriction site(s): none
Lost restriction site(s): none

Impact on splicing
Splice site type Wild type sequence CV Mutant type sequence CV Variation (%)
Donor
TTGgtacgt
84.5 _
TTGgtacat
72.3 _ *
-14.4 %

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD07WIL F0072 I0001ProbandNANAU.K.

Phenotypic groupDisease
NAUnknown

Clinical data


Symptom

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.