The UMD-FBN1 mutations database
Record ID: 525

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.8436dupp.Ser2813GlnfsX21HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AGCSerins1aFs.Stop at 2833Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
FibuCTDIII-like motif conserved AA fibu Glob DIII

Mutation impact


At the mRNA levelOn restriction map
One base duplicationNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
SWI01ZUR F0006 I01ProbandNANAUnknownSWITZERLAND

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
no clinical data

Reference


Reference IDPubMed IDReference
9811933199
Matyas G, De Paepe A, Halliday D, Boileau C, Pals G, Steinmann B. "Evaluation and application of denaturing HPLC for mutation detection in Marfan syndrome: Identification of 20 novel mutations and two novel polymorphisms in the FBN1 gene". Hum Mutat. 2002,19:443-56.