Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.3623delG | p.Cys1208LeufsX22 | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
TGT | Cys | del1b | Fs. | Stop at 1229 | Fr. |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like #15 | Disulfide bonds 1208-1221 (C2) |
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 |
BEL01GHE F0003 I01 | Proband | Male | familial | ? (7 years old) | BELGIUM |
Phenotypic group | Disease |
NA | Classical MFS |
Symptom | Severity |
C-Asc. aortic dilatation | |
C-Mitral valve prolapse | |
O-Astigmatism | |
S-Characteristic facial appearance | |
S-Chest deformity (unspecified) | |
S-High arched palate | |
S-Joint hypermobility (m) | |
S-Lordosis | severe |
S-Plain pes planus (M)(1) |
Reference ID | PubMed ID | Reference |
89 | 11700157 | Loeys B, Nuytinck L, Delvaux I, De Bie S, De Paepe A. "Genotype and phenotype analysis of 171 patients referred for molecular study of the fibrillin-1 gene FBN1 because of suspected Marfan syndrome". Arch Intern Med. 2001 Nov 12;161(20):2447-54. |