The UMD-FBN1 mutations database
Record ID: 554

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3228_3232dupp.Pro1078HisfsX12HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CCTProins5bFs.Stop at 1089Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #12 conserved AA in cbEGF-like

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
USA08PIT F0005 I01ProbandNANAUnknownU.S.A

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
no clinical data

Reference


Reference IDPubMed IDReference
4910533071
Yuan B, Thomas JP, von Kodolitsch Y, Pyeritz RE. "Comparison of heteroduplex analysis, direct sequencing, and enzyme mismatch cleavage for detecting mutations in a large gene, FBN1". Hum Mutat 1999;14(5):440-6 .