The UMD-FBN1 mutations database
Record ID: 5

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.6920G>Cp.Cys2307SerHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGCCysTCCSerG->CTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like#36 Disulfide bonds 2295-2307 (C3)NoNo

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)88 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA01BAL F0006 I01ProbandFemalefamilial? (53 years old)U.S.A

Phenotypic groupDisease
Type IV dClassical MFS

Clinical data


Symptom
C-Asc. aortic dilatation
C-Asc. aortic dissection
C-Mitral valve prolapse
S-Arachnodactyly (M)
S-Dolichostenomelia
S-Increased body length
S-Joint hypermobility (m)
S-Long bone over growth
S-Pectus carinatum (M)(2)
S-Pectus excavatum moderate (m)(1)
S-Scoliosis > 20° (M)(1)

Reference


Reference IDPubMed IDReference
31569206
Dietz HC, Pyeritz RE, Puffenberger EG, Kendzior RJ Jr, Corson GM, Maslen CL, Sakai LY, Francomano CA, Cutting GR. "Marfan phenotype variability in a family segregating a missense mutation in the epidermal growth factor-like motif of the fibrillin gene". J Clin Invest 1992 May;89(5):1674-80.