The UMD-FBN1 mutations database
Record ID: 209

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1585C>Tp.Arg529XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGAArgTGAStopC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #03 Ca2+ bindingYes, coding strandYes

Mutation impact


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

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA01BAL F0030 I01ProbandMalefamilialat 24 years oldU.S.A

Phenotypic groupDisease
Type II or IV ?Classical MFS

Clinical data


Symptom
C-Asc. aortic dilatation
C-Asc. aortic dissection
O-Myopia
S-Dolichostenomelia
S-Joint hypermobility (m)
S-Pectus carinatum (M)(2)
S-Plain pes planus (M)(1)
S-Scoliosis > 20° (M)(1)
SI-Significant striae atrophicae (m)(1)

Reference


Reference IDPubMed IDReference
609837823
Montgomery RA, Geraghty MT, Bull E, Gelb BD, Johnson M, McIntosh I, Francomano CA, Dietz HC. "Multiple molecular mechanisms underlying subdiagnostic variants of Marfan syndrome". Am J Hum Genet 1998 Dec;63(6):1703-11.