The UMD-FBN1 mutations database
Record ID: 893

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS63+5G>A (c.8051+5G>A)HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GGGGlyspl+5Spl.G->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #43 

Mutation impact


At the mRNA levelOn restriction map
Skipping of exon 63, frameshiftNew 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
AGGgtaagc
92 _
AGGgtaaac
79.9 _ *
-13.2 %

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD05LON F0030 I01ProbandNANAU.K.

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


SymptomAge
C-No cardiovascular involvement32

Reference


Reference IDPubMed IDReference
9617657824
Comeglio P, Johnson P, Arno G, Brice G, Evans A, Aragon-Martin J, da Silva FP, Kiotsekoglou A, Child A. "The importance of mutation detection in Marfan syndrome and Marfan-related disorders: report of 193 FBN1 mutations". Hum Mutat. 2007 Sep;28(9):928.