The UMD-FBN1 mutations database
Record ID: 411

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.364C>Tp.Arg122CysHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGCArgTGCCysC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
EGF-like #02 Yes, coding strandYes

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)
0.830.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
BEL01GHE F0046 I0916ProbandMalede novo15 years oldBELGIUM

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


Symptom
C-Mitral valve prolapse
O-Ectopia lentis
S-Arachnodactyly (M)
S-Arm span/height >1.05 (M)

Reference


Reference IDPubMed IDReference
8911700157
Loeys B, Nuytinck L, Delvaux I, De Bie S, De Paepe A. "Genotype and phenotype analysis of 171 patients referred for molecular study of the fibrillin-1 gene FBN1 because of suspected Marfan syndrome". Arch Intern Med. 2001 Nov 12;161(20):2447-54.