The UMD-FBN1 mutations database
Record ID: 1741

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3285C>Ap.Cys1095XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGCCysTGAStopC->ATv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #12 Disulfide bonds 1081-1095 (C4)NoNo

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 F0025 I0001ProbandNANAU.K.

Phenotypic groupDisease
NANA

Clinical data


Symptom
Clinical data will be implemented as soon as possible

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.