The UMD-FBN1 mutations database
Record ID: 435

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.7577delAp.Asn2526MetfsX156HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AATAsndel1bFs.Stop at 2681Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #40 Ca2+ binding

Mutation impact


At the mRNA levelOn restriction map
Deletion in a stretch of nucleotidesNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
BEL01GHE F0062 I1120ProbandFemalede novo50 years oldBELGIUM

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


Symptom
C-Asc. aortic dilatation
CF-Dolichocephaly
CF-Down-slanting palpebral fissures
CF-Malar hypoplasia
CF-Retrognathia
S-High arched palate
S-Plain pes planus (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.