Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.1421_1468del | p.Cys474_Asp490delinsTyr | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
TGT | Cys | del48b | InF | In frame del | InF |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
EGF-like #04 | Disulfide bonds 460-474 (C4) |
At the mRNA level | On restriction map |
No mechanism suspected | New restriction site(s): none Lost restriction site(s): none |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
FIN01HEL F0020 I01 | Proband | Male | de novo | ? (22 years old) | FINLAND |
Phenotypic group | Disease |
NA | Classical MFS |
Symptom |
C-Mitral valve prolapse |
C-Tricuspid valve prolapse |
O-Ectopia lentis |
O-Myopia |
S-Arachnodactyly (M) |
S-High arched palate |
S-Joint hypermobility (m) |
S-Scoliosis > 20° (M)(1) |
SI-Inguinal hernia |
SI-Significant striae atrophicae (m)(1) |
Reference ID | PubMed ID | Reference |
68 | - | Rantamaki T, Peltonnen L (Personal communication 2000). |