Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.7926delC | p.Phe2642LeufsX40 | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
TTC | Phe | del1c | Fs. | Stop at 2681 | Fr. |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like #42 |
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 |
UKD05LON F0006 I26 | Proband | Female | de novo | 5 years old | U.K. |
Phenotypic group | Disease |
NA | Classical MFS |
Symptom | Severity |
C-Mitral valve prolapse | surgery |
O-Ectopia lentis | |
O-Increased axial length of globe (m) | |
O-Iridodonesis | |
O-Myopia | |
S-Arachnodactyly (M) | |
S-Chest deformity (unspecified) | |
S-Foot deformity | |
S-High arched palate | |
S-Plain pes planus (M)(1) | |
S-Reduced US/LS ratio <0.87 (M) | |
SI-Significant striae atrophicae (m)(1) |
Reference ID | PubMed ID | Reference |
83 | 11748851 | Comeglio P, Evans AL, Brice GW and Child AH. "Detection of six novel FBN1 mutations in british patients affected by Marfan syndrome". Human Mutation, 2001, 18(6): 546-7. |