The UMD-FBN1 mutations database
Record ID: 9

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.5134_5137dupp.Asn1713IlefsX23HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AACAsnins4bFs.Stop at 1735Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP#05 N-linked glycosylation

Mutation impact


At the mRNA levelOn restriction map
Duplication of a repeated sequenceNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA01BAL F0009 I03ProbandMalede novo? U.S.A

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


SymptomSeverity
C-Asc. aortic dilatationborderline
C-Mitral valve prolapse
O-Myopia
S-Dolichostenomeliasevere
S-Long bone over growthmoderate
SI-Significant striae atrophicae (m)(1)

Reference


Reference IDPubMed IDReference
48406497
Dietz HC, McIntosh I, Sakai LY, Corson GM, Chalberg SC, Pyeritz RE, Francomano CA. "Four novel FBN1 mutations: significance for mutant transcript level and EGF-like domain calcium binding in the pathogenesis of Marfan syndrome". Genomics 1993 Aug;17(2):468-75.