The UMD-FBN1 mutations database
Record ID: 381

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.5743C>Ap.Arg1915SerHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGCArgAGCSerC->ATv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #28 Ca2+ bindingYes, coding strandNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Alu I, Pst I, Pvu II, Sfe I
Lost restriction site(s): none

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

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
BEL01GHE F0017 I2066ProbandMalede novo3,5 years oldBELGIUM

Phenotypic groupDisease
NADominant ectopia lentis

Clinical data


Symptom
O-Ectopia lentis

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.