The UMD-FBN1 mutations database
Record ID: 3017

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
NET03LEI F0004 I0001ProbandNANANETHERLAND

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom

Reference


Reference IDPubMed IDReference
21920886638
Hilhorst-Hofstee Y, Rijlaarsdam ME, Scholte AJ, Swart-van den Berg M, Versteegh MI, van der Schoot-van Velzen I, Sch*bitz HJ, Bijlsma EK, Baars MJ, Kerstjens-Frederikse WS, Giltay JC, Hamel BC, Breuning MH, Pals G. "The clinical spectrum of missense mutations of the first aspartic acid of cbEGF-like domains in fibrillin-1 including a recessive family". Hum Mutat. 2010 Dec;31(12):E1915-27.