The UMD-FBN1 mutations database
Record ID: 69

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3217G>Ap.Glu1073LysHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GAAGluAAALysG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #12 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)76 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA05HOU F0010 I269ProbandFemalede novoat birth1 weekU.S.A

Phenotypic groupDisease
NANeonatal MFS

Clinical data


Symptom
C-Aortic insufficiency
C-Asc. aortic dilatation
C-Mitral regurgitation
C-Pulmonary valve insufficiency
S-Abnormal ears
S-Arachnodactyly (M)
S-Characteristic facial appearance
S-Joint limitations

Reference


Reference IDPubMed IDReference
268882780
Putnam EA, Cho M, Zinn AB, Towbin JA, Byers PH, Milewicz DM. "Delineation of the Marfan phenotype associated with mutations in exons 23-32 of the FBN1 gene". Am J Med Genet 1996 Mar 29;62(3):233-42.