The UMD-FBN1 mutations database
Record ID: 534

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1711G>Tp.Glu571XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GAAGluTAAStopG->TTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #04 Ca2+ bindingYes, non coding strandNo

Mutation impact


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

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA04NOR F0003 I1650ProbandNAfamilialUnknownU.S.A

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
no clinical data

Reference


Reference IDPubMed IDReference
10111826022
Korkko J, Kaitila I, Lonnqvist L, Peltonen L, Ala-Kokko L. "Sensitivity of conformation sensitive gel electrophoresis in detecting mutations in Marfan syndrome and related conditions". J Med Genet. 2002 Jan;39(1):34-41.