The UMD-FBN1 mutations database
Record ID: 286

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.4485delCp.Thr1496ArgfsX24HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ACCThrdel1cFs.Stop at 1519Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #22 

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
UKD04OXF F0004 I04ProbandNAfamilial? (31 years old)U.K.

Phenotypic groupDisease
NAClassical MFS +

Clinical data


Symptom
C-Asc. aortic dilatation
C-Mitral valve prolapse
S-Arachnodactyly (M)
S-Arm span/height >1.05 (M)
S-Chest deformity (unspecified)
S-High arched palate
S-Scoliosis > 20° (M)(1)
SI-Significant striae atrophicae (m)(1)

Reference


Reference IDPubMed IDReference
5810647894
Halliday D, Hutchinson S, Kettle S, Firth H, Wordsworth P, Handford PA. "Molecular analysis of eight mutations in FBN1". Hum Genet 1999 Dec;105(6):587-97.