The UMD-FBN1 mutations database
Record ID: 2127

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.4467T>Ap.Asn1489LysHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AATAsnAAALysT->ATv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #22 Ca2+ bindingNoNo

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)
10.03 (pathogenous)92 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA05HOU F0019 I0001ProbandFemaleNAU.S.A.

Phenotypic groupDisease
NATAAD

Clinical data


SymptomAge
C-Asc. aortic dilatation56
C-Desc. aortic dissection (thor. or abdo.)56
S-High arched palate56

Reference


Reference IDPubMed IDReference
19320082464
Brautbar A, LeMaire SA, Franco LM, Coselli JS, Milewicz DM, Belmont JW. "FBN1 mutations in patients with descending thoracic aortic dissections". Am J Med Genet A. 2010 Feb;152A(2):413-6.