The UMD-FBN1 mutations database
Record ID: 559

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1633C>Tp.Arg545CysHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGCArgTGCCysC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #04 NoYes

Mutation impact


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

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.670.00 (pathogenous)94 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA04NOR F0013 I3675ProbandMalefamilial9 years oldU.S.A

Phenotypic groupDisease
NAClassical MFS

Clinical data


SymptomSeverity
C-Mitral regurgitation
O-Ectopia lentisbilateral
S-Arachnodactyly (M)
S-Crowding teeth (m)
S-High arched palate
S-Joint hypermobility (m)
S-Plain pes planus (M)(1)
S-Reduced US/LS ratio <0.87 (M)

Reference


Reference IDPubMed IDReference
102-
Hyland JC (Personal communication 2003).