The UMD-FBN1 mutations database
Record ID: 3354

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1586G>Ap.Arg529GlnHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGAArgCAAGlnG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #03 Ca2+ bindingYes, non coding strandYes

Mutation impact


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

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.830.00 (pathogenous)82 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
ITA01FLO F0110 I0001ProbandMaleNA15ITALIA

Phenotypic groupDisease
NANA

Clinical data


SymptomAge
C-Aortic insufficiency15
C-Bicuspid aortic valve15

Reference


Reference IDPubMed IDReference
28224564502
Pepe G, Nistri S, Giusti B, Sticchi E, Attanasio M, Porciani C, Abbate R, Bonow RO, Yacoub M, Gensini GF. "Identification of fibrillin 1 gene mutations in patients with bicuspid aortic valve (BAV) without Marfan syndrome". BMC Med Genet. 2014 Feb 24;15:23.