The UMD-FBN1 mutations database
Record ID: 3212

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3519C>Gp.Asn1173LysHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AACAsnAAGLysC->GTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #14 Ca2+ bindingYes, coding strandNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Alu I
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.670.00 (pathogenous)88 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
CHI16TIA F0001 I0001ProbandNAfamilialCHINA

Phenotypic groupDisease
NADominant ectopia lentis

Clinical data


Symptom
O-Ectopia lentis

Reference


Reference IDPubMed IDReference
26023141514
Feng XL, Li ND, Wang LM, Wang YC, Zhao KX. [Recurrent N1173K mutation in FBN1 gene in a Chinese family with ectopia lentis]. Zhonghua Yan Ke Za Zhi. 2012 Aug;48(8):728-32.