The UMD-FBN1 mutations database
Record ID: 1863

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.965C>Gp.Ser322CysHomozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TCTSerTGTCysC->GTv

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

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)
0.830.02 (pathogenous)69 (Probably pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA11DUR F0001 I0001ProbandMalefamilial? (36 years old)U.S.A.

Phenotypic groupDisease
NADominant ectopia lentis

Clinical data


SymptomSeverityAge
O-Corneal guttae36
O-Ectopia lentisbilateral8
O-Glaucoma8
S-Chest deformity (unspecified)36
S-High arched palate36
S-Joint limitations36

Reference


Reference IDPubMed IDReference
16116971892
Challa P, Hauser MA, Luna CC, Freedman SF, Pericak-Vance M, Yang J, McDonald MT, Allingham RR. "Juvenile bilateral lens dislocation and glaucoma associated with a novel mutation in the fibrillin 1 gene". Mol Vis. 2006 Aug 28;12:1009-15.