The UMD-FBN1 mutations database
Record ID: 1864

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.184C>Tp.Arg62CysHomozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGTArgTGTCysC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
4-cys motif LTBP-like NoYes

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Nla III
Lost restriction site(s): Mae II

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
CHI07SHA F0001 I0001ProbandFemalefamilial?CHINA

Phenotypic groupDisease
NADominant ectopia lentis

Clinical data


Symptom
O-Ectopia lentis

Reference


Reference IDPubMed IDReference
16216765689
Yu R, Lai Z, Zhou W, Ti DD, Zhang XN. "Recurrent FBN1 mutation (R62C) in a Chinese family with isolated ectopia lentis". Am J Ophthalmol. 2006 Jun;141(6):1136-8.