The UMD-FBN1 mutations database
Record ID: 3348

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.2051G>Ap.Cys684TyrHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGTCysTATTyrG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP#02 C in disulfide bonds 684-699NoNo

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
GER07COL F0005 I0001ProbandFemalefamilialSAUDI ARABIA

Phenotypic groupDisease
NALens subluxation

Clinical data


SymptomAge
O-Ectopia lentis4

Reference


Reference IDPubMed IDReference
28124698609
Khan AO, Bolz HJ, Bergmann C. "Results of fibrillin-1 gene analysis in children from inbred families with lens subluxation". J AAPOS. 2014 Apr;18(2):134-9.