The UMD-FBN1 mutations database
Record ID: 283

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.7180C>Tp.Arg2394XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGAArgTGAStopC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP #07 Yes, coding strandYes

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): Hae III

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD04OXF F0008 I08ProbandNAde novo? (43 years old)U.K.

Phenotypic groupDisease
Type I or II ?Classical MFS

Clinical data


SymptomSeverity
C-Asc. aortic dissection
L-Spontaneous pneumothorax
O-Ectopia lentis
S-Arachnodactyly (M)
S-Characteristic facial appearance
S-High arched palate
S-Pectus excavatum severesurgery
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.