The UMD-FBN1 mutations database
Record ID: 1727

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1791_1792dupp.Cys598PhefsX28HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGCCysins2bFs.Stop at 625Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #05 Disulfide bonds 598-611 (C5)

Mutation impact


At the mRNA levelOn restriction map
Duplication in a stretch of nucleotidesNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD07WIL F0011 I0001ProbandNANAU.K.

Phenotypic groupDisease
NANA

Clinical data


Symptom

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.