Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.7606G>A | p.Gly2536Arg | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
GGG | Gly | AGG | Arg | G->A | Ts |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like #40 | Ca2+ binding | Yes, non coding strand | 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) |
---|---|---|
1 | 0.00 (pathogenous) | 100 (Pathogenous) |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
UKD05LON F0005 I24 | Proband | Female | familial | 46 years old | U.K. |
Phenotypic group | Disease |
NA | Incomplete MFS |
Symptom |
C-Asc. aortic dilatation |
C-Mitral regurgitation |
C-Mitral valve prolapse |
L-Pulmonary emphysema |
O-Myopia |
S-Arachnodactyly (M) |
S-Arm span/height >1.05 (M) |
S-Chondromalacia Patellae |
S-Joint hypermobility (m) |
S-Kyphosis |
S-Plain pes planus (M)(1) |
S-Reduced US/LS ratio <0.87 (M) |
SI-Tendency to ecchymosis |
SI-Varicose veins |
SI-Varicose veins |
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. |