The UMD-FBN1 mutations database
Record ID: 1721

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.772C>Tp.Gln258XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CAGGlnTAGStopC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #01 Yes, coding strandNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD07WIL F0005 I0001ProbandMalefamilial? (35 years old)U.K.

Phenotypic groupDisease
NAClassical MFS

Clinical data


SymptomAge
C-Asc. aortic dilatation35
S-Incomplete description35
SI-Incomplete description35

Reference


Reference IDPubMed IDReference
15717627385
Howarth R, Yearwood C, Harvey JF. "Application of dHPLC for mutation detection of the fibrillin-1 gene for the diagnosis of Marfan syndrome in a National Health Service Laboratory". Genet Test. 2007 Summer;11(2):146-52.