The UMD-FBN1 mutations database
Record ID: 413

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.7852G>Ap.Gly2618ArgHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GGAGlyAGAArgG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #42 Yes, non coding strandYes

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Pst I, Sfe I
Lost restriction site(s): none

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

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
BEL01GHE F0048 I1427ProbandFemalefamilial15 yeas oldBELGIUM

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


Symptom
CF-Dolichocephaly
CF-Down-slanting palpebral fissures
CF-Malar hypoplasia
S-Arachnodactyly (M)
S-Arm span/height >1.05 (M)
S-High arched palate
S-Plain pes planus (M)(1)
S-Reduced US/LS ratio <0.87 (M)

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.