The UMD-FBN1 mutations database
Record ID: 414

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1678G>Ap.Gly560SerHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GGCGlyAGCSerG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #04 conserved AA in cbEGF-likeYes, non 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
BEL01GHE F0049 I1115ProbandFemalefamilial3 years oldBELGIUM

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Asc. aortic dilatation
C-Mitral valve prolapse
O-Ectopia lentis
S-Chest deformity (unspecified)
S-Joint hypermobility (m)
S-Plain pes planus (M)(1)
S-Scoliosis > 20° (M)(1)

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.