The UMD-FBN1 mutations database
Record ID: 338

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.4615C>Tp.Arg1539XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGAArgTGAStopC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP#04 Yes, coding strandYes

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): Taq I

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
GER01BER F0013 I27ProbandMalefamilialGERMANY

Phenotypic groupDisease
NAClassical MFS

Clinical data


SymptomSeverity
C-Asc. aortic dilatation
C-Asc. aortic dissectionsurgery
CF-Dolichocephaly
L-Spontaneous pneumothorax
O-Ectopia lentis
O-Myopia
S-Chest deformity (unspecified)
S-High arched palate

Reference


Reference IDPubMed IDReference
8011175294
Tiecke F, Katzke S, Booms P, Robinson P, Neumann L, Godfrey M, Mathews KR, Scheuner M, Hinkel GK, Brenner RE, H*vels-GŸrich H, Hagemeier C, Fuchs J, Skovby F, Rosenberg T. "Classic , early-onset severe and neonatal Marfan syndrome : Thirteen mutations and genotype-phenotype correlations in FBN1 exons 24-40". Eur J Hum Genet 2001, 9:13-21.