Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.4930C>T | p.Arg1644X | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
CGA | Arg | TGA | Stop | C->T | Ts |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like #23 | No | Yes |
At the mRNA level | On restriction map |
NA | New restriction site(s): Nla III Lost restriction site(s): none |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
AUS01NAD F0020 I08 | Proband | Female | de novo | at 13 years old | AUSTRALIA |
Phenotypic group | Disease |
NA | Classical MFS |
Symptom |
C-Asc. aortic dilatation |
C-Asc. aortic dilatation |
O-Coloboma |
S-Arachnodactyly (M) |
S-Characteristic facial appearance |
S-Crowding teeth (m) |
S-High arched palate |
S-Joint limitations |
SI-Significant striae atrophicae (m)(1) |
Reference ID | PubMed ID | Reference |
114 | 14695540 | Biggin A, Holman K, Brett M, Bennetts B, Ades L. "Detection of thirty novel FBN1 mutations in patients with Marfan syndrome or a related fibrillinopathy". Hum Mutat. 2004 Jan;23(1):99. |