Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.1633C>T | p.Arg545Cys | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
CGC | Arg | TGC | Cys | C->T | Ts |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like #04 | No | Yes |
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) |
---|---|---|
0.67 | 0.00 (pathogenous) | 94 (Pathogenous) |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
UKD01EDI F0006 I01 | Proband | Female | familial | ? (33 years old) | U.K. |
Phenotypic group | Disease |
NA | Classical MFS |
Symptom |
C-Desc. aortic dilatation (thor or abdo) |
O-Cataract |
O-Ectopia lentis |
O-Retinal detachment |
S-Arachnodactyly (M) |
S-Characteristic facial appearance |
S-Chest deformity (unspecified) |
S-Joint limitations |
Reference ID | PubMed ID | Reference |
41 | 9338581 | Hayward C, Porteous ME, Brock DJ. "Mutation screening of all 65 exons of the fibrillin-1 gene in 60 patients with Marfan syndrome: report of 12 novel mutations". Hum Mutat 1997;10(4):280-9 . |