The UMD-FBN1 mutations database
Record ID: 2354

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS36+2T>C (c.4582+2T>C)HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GATAspspl+2Spl.T->CTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP#04 conserved AA in TGFBP

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
TTGgtaaga
90.9 _
TTGgcaaga
64.1 _ *
-29.5 %

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
FRA01BOU F0611 I0648ProbandMalefamilialFRANCE

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


Symptom

Reference


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