Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.1709G>A | p.Cys570Tyr | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
TGT | Cys | TAT | Tyr | G->A | Ts |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like #04 | Disulfide bonds 557-570 (C6) | No | No |
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) | 100 (Pathogenous) |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
BEL01GHE F0019 I2264 | Proband | Male | familial | 36 years old | BELGIUM |
Phenotypic group | Disease |
NA | Classical MFS |
Symptom |
CF-Dolichocephaly |
CF-Malar hypoplasia |
O-Ectopia lentis |
S-Arachnodactyly (M) |
S-Chest deformity (unspecified) |
S-High arched palate |
S-Lordosis |
S-Plain pes planus (M)(1) |
S-Reduced US/LS ratio <0.87 (M) |
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. |