The UMD-FBN1 mutations database
Record ID: 750

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3419G>Tp.Cys1140PheHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGCCysTTCPheG->TTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #13 Disulfide bonds 1140-1153 (C5)NoNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): EcoR I
Lost restriction site(s): BspW I

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.670.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
BEL01GHE F0135 I01ProbandNANABELGIUM

Phenotypic groupDisease
NANeonatal MFS

Clinical data


Symptom

Reference


Reference IDReference
122Unpublished data