The UMD-FBN1 mutations database
Record ID: 2994

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3464A>Gp.Asp1155GlyHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GACAspGGCGlyA->GTs

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

Mutation impact


At the mRNA levelOn restriction map
First nucleotide of the exon, cDNA not testedNew restriction site(s): none
Lost restriction site(s): Taq I

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

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD05LON F0199 I0001ProbandNAde novoU.K.

Phenotypic groupDisease
NADominant ectopia lentis

Clinical data


Symptom
no clinical data

Reference


Reference IDPubMed IDReference
23822736615
Chandra A, Aragon-Martin JA, Hughes K, Gati S, Reddy MA, Deshpande C, Cormack G, Child AH, Charteris DG, Arno G. "A genotype-phenotype comparison of ADAMTSL4 and FBN1 in isolated ectopia lentis". Invest Ophthalmol Vis Sci. 2012 Jul 24;53(8):4889-96.