The UMD-FBN1 mutations database
Record ID: 526

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS11+2T>C (c.1468+2T>C)HeterozygousMutation

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

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #03 Ca2+ binding

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
TTGgtacgt
84.5 _
TTGgcacgt
57.6 _ *
-31.8 %

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
SWI01ZUR F0001 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.