The UMD-FBN1 mutations database
Record ID: 369

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.7926delCp.Phe2642LeufsX40HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TTCPhedel1cFs.Stop at 2681Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #42 

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
UKD05LON F0006 I26ProbandFemalede novo5 years oldU.K.

Phenotypic groupDisease
NAClassical MFS

Clinical data


SymptomSeverity
C-Mitral valve prolapsesurgery
O-Ectopia lentis
O-Increased axial length of globe (m)
O-Iridodonesis
O-Myopia
S-Arachnodactyly (M)
S-Chest deformity (unspecified)
S-Foot deformity
S-High arched palate
S-Plain pes planus (M)(1)
S-Reduced US/LS ratio <0.87 (M)
SI-Significant striae atrophicae (m)(1)

Reference


Reference IDPubMed IDReference
8311748851
Comeglio P, Evans AL, Brice GW and Child AH. "Detection of six novel FBN1 mutations in british patients affected by Marfan syndrome". Human Mutation, 2001, 18(6): 546-7.