The UMD-FBN1 mutations database
Record ID: 2341

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1957_1958delGTp.Val653XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GTTValdel2aFs.Stop at 653Fr.

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

Mutation impact


At the mRNA levelOn restriction map
Deletion of a repeated sequenceNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
FRA01BOU F0597 I0634ProbandFemalefamilialFRANCE

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom

Reference


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