Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.4467T>A | p.Asn1489Lys | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
AAT | Asn | AAA | Lys | T->A | Tv |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like #22 | Ca2+ binding | 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 | 0.03 (pathogenous) | 92 (Pathogenous) |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
USA05HOU F0019 I0001 | Proband | Female | NA | U.S.A. |
Phenotypic group | Disease |
NA | TAAD |
Symptom | Age |
C-Asc. aortic dilatation | 56 |
C-Desc. aortic dissection (thor. or abdo.) | 56 |
S-High arched palate | 56 |
Reference ID | PubMed ID | Reference |
193 | 20082464 | Brautbar A, LeMaire SA, Franco LM, Coselli JS, Milewicz DM, Belmont JW. "FBN1 mutations in patients with descending thoracic aortic dissections". Am J Med Genet A. 2010 Feb;152A(2):413-6. |