The UMD-FBN1 mutations database
Record ID: 437

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.390delTp.Ser130ArgfsX12HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AGTSerdel1cFs.Stop at 141Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
EGF-like #02 

Mutation impact


At the mRNA levelOn restriction map
No mechanism suspectedNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
BEL01GHE F0064 I1548ProbandMalede novoat birthBELGIUM

Phenotypic groupDisease
NANeonatal MFS

Clinical data


Symptom

Reference


Reference IDPubMed IDReference
8911700157
Loeys B, Nuytinck L, Delvaux I, De Bie S, De Paepe A. "Genotype and phenotype analysis of 171 patients referred for molecular study of the fibrillin-1 gene FBN1 because of suspected Marfan syndrome". Arch Intern Med. 2001 Nov 12;161(20):2447-54.