The UMD-FBN1 mutations database
Record ID: 2734

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS50+1G>A (c.6313+1G>A)HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GAGGluspl+1Spl.G->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP#06 

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
ATGgtatgt
85.5 _
ATGatatgt
58.7 _ *
-31.4 %

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
FRA01BOU F1001 I1037ProbandFemaleNAFRANCE

Phenotypic groupDisease
NAUnknown

Clinical data


Symptom

Reference


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