The UMD-FBN1 mutations database
Record ID: 2997

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3344A>Gp.Asp1115GlyHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GATAspGGTGlyA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #13 Ca2+ bindingYes, non coding strandNo

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
UKD05LON F0202 I0001ProbandFemaleNAU.K.

Phenotypic groupDisease
NAMFS

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.