The UMD-FBN1 mutations database
Record ID: 1871

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.2860C>Tp.Arg954CysHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGCArgTGCCysC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP#03 conserved AA in TGFBPYes, 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
CHI10BEI F0001 I0001ProbandMalefamilial37CHINA

Phenotypic groupDisease
NADominant ectopia lentis

Clinical data


SymptomAge
O-Ectopia lentis37
O-Glaucoma37

Reference


Reference IDPubMed IDReference
16618615205
Deng T, Dong B, Zhang X, Dai H, Li Y. "Late-onset bilateral lens dislocation and glaucoma associated with a novel mutation in FBN1". Mol Vis. 2008 Jun 30;14:1229-33.