The UMD-FBN1 mutations database
Record ID: 1670

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS29+4A>T (c.3712+4A>T)HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GACAspspl+4Spl.A->TTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #16 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
CCGgtgagt
94.1 _
CCGgtgtgt
85.3 _
-9.4 %

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
CHI06FUJ F0002 I0001ProbandNANACHINA

Phenotypic groupDisease
NAUnknown

Clinical data


Symptom
Clinical data will be implemented as soon as possible

Reference


Reference IDPubMed IDReference
15617680538
Chen XJ, Wu YA, Chen FW, Chen FL, Huang Y, Huang XL, Ma XN, Chen T. [Two gene mutations in fibrillin 1 of Marfan syndrome]. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2007 Aug;24(4):440-2.