The UMD-FBN1 mutations database
Record ID: 2194

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS1+2dup2dupT (c.164+2dup2dupT)p.Met1?HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ATGMetspl+2Spl.dup2dupT

StructureKey Residue (HCD)Pyrimidin doubletCpG
Signal peptide 

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
AGGgtaaag
80,1 _
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Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
FRA01BOU F0450 I0487ProbandFemalede novoFRANCE

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom

Reference


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