The UMD-FBN1 mutations database
Record ID: 614

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.718C>Tp.Arg240CysHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGCArgTGCCysC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
Hybrid module#01 Yes, 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)
0.670.00 (pathogenous)94 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD05LON F0016 I01ProbandMaleNA(56 years old)U.K.

Phenotypic groupDisease
NADominant ectopia lentis

Clinical data


Symptom
O-Ectopia lentis
O-Myopia
O-Strabismus
S-Joint hypermobility (m)
SI-Inguinal hernia
SI-Significant striae atrophicae (m)(1)
SI-Skin hyperextensibility

Reference


Reference IDPubMed IDReference
10812446365
Comeglio P, Evans AL, Brice G, Cooling RJ, Child AH. "Identification of FBN1 gene mutations in patients with ectopia lentis and marfanoid habitus". Br J Ophthalmol. 2002 Dec;86(12):1359-62.