The UMD-FBN1 mutations database
Record ID: 29

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3464_3480delp.Asp1155AlafsX32HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GACAspdel17bFs.Stop at 1186Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #14 Ca2+ binding

Mutation impact


At the mRNA levelOn restriction map
No mechanism suspectedNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA03STA F0003 I773ProbandNAfamilialU.S.A

Phenotypic groupDisease
Type IIIncomplete MFS

Clinical data


SymptomSeverity
C-Asc. aortic dilatationsurgery
C-Mitral valve prolapse
S-Increased body length
S-Long bone over growth
S-Pectus excavatum moderate (m)(1)

Reference


Reference IDPubMed IDReference
128281141
Tynan K, Comeau K, Pearson M, Wilgenbus P, Levitt D, Gasner C, Berg MA, Miller DC, Francke U. "Mutation screening of complete fibrillin-1 coding sequence: report of five new mutations, including two in 8-cysteine domains". Hum Mol Genet 1993 Nov;2(11):1813-21.