The UMD-FBN1 mutations database
Record ID: 64

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3215A>Gp.Asp1072GlyHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GACAspGGCGlyA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #12 Ca2+ bindingYes, non coding strandNo

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)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA05HOU F0009 I182ProbandNAde novoU.S.A

Phenotypic groupDisease
NANeonatal MFS

Clinical data


SymptomSeverity
C-Asc. aortic dilatation
C-Asc. aortic dilatationsurgery
C-Mitral regurgitation
C-Mitral valve prolapse
C-Pulmonary art. dilatation
CF-Dolichocephaly
CF-Micrognathia
O-Iridodonesis
O-Myopia
S-Abnormal ears
S-Arachnodactyly (M)
S-Characteristic facial appearance
S-Joint hypermobility (m)
S-Joint limitations
S-Pectus excavatum moderate (m)(1)

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.