The UMD-FBN1 mutations database
Record ID: 2713

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS14+5G>A (c.1837+5G>A)HeterozygousMutation

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

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #06 Ca2+ binding

Mutation impact


At the mRNA levelOn restriction map
Not tested on cDNANew 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
AAGgttcgt
85.2 _
AAGgttcat
73.1 _ *
-14.3 %

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
FRA01BOU F0979 I1015ProbandMaleNAFRANCE

Phenotypic groupDisease
NAMFS

Clinical data


Symptom

Reference


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