The UMD-FBN1 mutations database
Record ID: 2375

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1347_1348dupp.Thr450IlefsX130HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ACTThrins2bFs.Stop at 579

StructureKey Residue (HCD)Pyrimidin doubletCpG
EGF-like #04 N-Term MMPs site

Mutation impact


At the mRNA levelOn restriction map
Duplication 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 F0632 I0669ProbandFemalede novoFRANCE

Phenotypic groupDisease
NAIsolated skeletal features

Clinical data


Symptom

Reference


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