The UMD-FBN1 mutations database
Record ID: 137

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3623delGp.Cys1208LeufsX22HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGTCysdel1bFs.Stop at 1229Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #15 Disulfide bonds 1208-1221 (C2)

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 F0003 I01ProbandMalefamilial? (7 years old)BELGIUM

Phenotypic groupDisease
NAClassical MFS

Clinical data


SymptomSeverity
C-Asc. aortic dilatation
C-Mitral valve prolapse
O-Astigmatism
S-Characteristic facial appearance
S-Chest deformity (unspecified)
S-High arched palate
S-Joint hypermobility (m)
S-Lordosissevere
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.