The UMD-FBN1 mutations database
Record ID: 1865

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.640G>Ap.Gly214SerHomozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GGCGlyAGCSerG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
Hybrid module#01 Yes, non coding strandYes

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): Eag I, Hae III

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.670.00 (pathogenous)76 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
CHI08BEI F0001 I0001ProbandNAfamilial?CHINA

Phenotypic groupDisease
NADominant ectopia lentis

Clinical data


Symptom
O-Ectopia lentis

Reference


Reference IDPubMed IDReference
16415733436
Sui RF, Wei HB, Zhao JL, Hu SY, Wang B, Huang SZ, Dong M. [Novel mutation of fibrillin 1 gene cause ectopia lentis in a Chinese family]. Zhonghua Yan Ke Za Zhi. 2004 Dec;40(12):828-31.