Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.4270C>G | p.Pro1424Ala | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
CCA | Pro | GCA | Ala | C->G | Tv |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like #20 | Ca2+ binding | Yes, coding strand | 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) |
---|---|---|
1 | 0.25 (non pathogenous) | 71 (Probably pathogenous) |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
AUS01NAD F0008 I01 | Proband | Male | familial | ? (2,5 years old) | AUSTRALIA |
Phenotypic group | Disease |
NA | Incomplete MFS |
Symptom | Severity |
O-Divergent strabismus | |
O-Myopia | early |
S-Arachnodactyly (M) | |
S-Characteristic facial appearance | |
S-Joint hypermobility (m) |
Reference ID | PubMed ID | Reference |
114 | 14695540 | Biggin A, Holman K, Brett M, Bennetts B, Ades L. "Detection of thirty novel FBN1 mutations in patients with Marfan syndrome or a related fibrillinopathy". Hum Mutat. 2004 Jan;23(1):99. |