Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.6920G>C | p.Cys2307Ser | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
TGC | Cys | TCC | Ser | G->C | Tv |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like#36 | Disulfide bonds 2295-2307 (C3) | No | No |
At the mRNA level | On restriction map |
NA | New restriction site(s): none Lost restriction site(s): none |
Conservation (0-1) | SIFT (0-1) | UMD-predictor (0-100) |
---|---|---|
0.83 | 0.00 (pathogenous) | 88 (Pathogenous) |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
USA01BAL F0006 I01 | Proband | Female | familial | ? (53 years old) | U.S.A |
Phenotypic group | Disease |
Type IV d | Classical MFS |
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 ID | PubMed ID | Reference |
3 | 1569206 | 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 . |