The UMD-FBN1 mutations database
Record ID: 2869

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3676G>Tp.Gly1226XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GGAGlyTGAStopG->TTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #15 conserved AA in cbEGF-likeYes, non coding strandNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): BstK I, Dsa V, Hpa II, Msp I, ScrF I

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
POR01POR F0006 I0001ProbandMalefamilialPORTUGAL

Phenotypic groupDisease
NAMFS

Clinical data


SymptomAge
C-Asc. aortic dilatation24
C-Mitral valve prolapse24

Reference


Reference IDPubMed IDReference
22422005308
Lebreiro A, Martins E, Cruz C, Almeida J, Pimenta S, Bernardes M, Carlos Machado J, Jœlia Maciel M, Abreu-Lima C. [Genotypic characterization of a Portuguese population of Marfan syndrome patients]. Rev Port Cardiol. 2011 Jul;30(7-8):649-54.