Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.965C>G | p.Ser322Cys | Homozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
TCT | Ser | TGT | Cys | C->G | Tv |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like #02 | 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) |
---|---|---|
0.83 | 0.02 (pathogenous) | 69 (Probably pathogenous) |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
USA11DUR F0001 I0001 | Proband | Male | familial | ? (36 years old) | U.S.A. |
Phenotypic group | Disease |
NA | Dominant ectopia lentis |
Symptom | Severity | Age |
O-Corneal guttae | 36 | |
O-Ectopia lentis | bilateral | 8 |
O-Glaucoma | 8 | |
S-Chest deformity (unspecified) | 36 | |
S-High arched palate | 36 | |
S-Joint limitations | 36 |
Reference ID | PubMed ID | Reference |
161 | 16971892 | Challa P, Hauser MA, Luna CC, Freedman SF, Pericak-Vance M, Yang J, McDonald MT, Allingham RR. "Juvenile bilateral lens dislocation and glaucoma associated with a novel mutation in the fibrillin 1 gene". Mol Vis. 2006 Aug 28;12:1009-15. |