Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.8057G>T | p.Cys2686Phe | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
TGT | Cys | TTT | Phe | G->T | Tv |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like #43 | Disulfide bonds 2674-2686 (C6) | 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) |
---|---|---|
1 | 0.00 (pathogenous) | 100 (Pathogenous) |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
USA03STA F0049 I05 | Proband | NA | NA | ? (27 years old) | U.S.A |
Phenotypic group | Disease |
Type IV d | Classical MFS |
Symptom |
C-Aortic surgery |
C-Asc. aortic dilatation |
O-Myopia |
S-Arachnodactyly (M) |
S-High arched palate |
S-Joint limitations |
S-Pectus carinatum (M)(2) |
SI-Significant striae atrophicae (m)(1) |
Reference ID | PubMed ID | Reference |
54 | 10464652 | Liu WO, Oefner PJ, Qian C, Odom RS, Francke U. "Denaturing HPLC-identified novel FBN1 mutations, polymorphisms, and sequence variants in Marfan syndrome and related connective tissue disorders". Genet Test 1997-98;1(4):237-42. |