The UMD-FBN1 mutations database
Record ID: 436

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.124delGp.Ala42ProfsX66HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GCCAladel1aFs.Stop at 107Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
NH2 unique region N-Term Furine/Pace site

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 F0063 I1656ProbandMalede novo19 years oldBELGIUM

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Asc. aortic dilatation
CNS-Lumbosacral dural ectasia
S-Arachnodactyly (M)
S-Chest deformity (unspecified)
S-Joint hypermobility (m)
S-Plain pes planus (M)(1)
S-Reduced US/LS ratio <0.87 (M)
S-Scoliosis > 20° (M)(1)
SI-Significant striae atrophicae (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.