Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.IVS11+2T>C (c.1468+2T>C) | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
GAT | Asp | spl+2 | Spl. | T->C | Ts |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like #03 | Ca2+ binding |
At the mRNA level | On restriction map |
Not tested on cDNA | New restriction site(s): none Lost restriction site(s): none |
Impact on splicing | ||||||||||
Splice site type | Wild type sequence | CV | Mutant type sequence | CV | Variation (%) | |||||
TTGgtacgt |
| TTGgcacgt |
| -31.8 % |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
SWI01ZUR F0001 I01 | Proband | NA | NA | Unknown | SWITZERLAND |
Phenotypic group | Disease |
NA | Classical MFS |
Symptom |
no clinical data |
Reference ID | PubMed ID | Reference |
98 | 11933199 | Matyas G, De Paepe A, Halliday D, Boileau C, Pals G, Steinmann B. "Evaluation and application of denaturing HPLC for mutation detection in Marfan syndrome: Identification of 20 novel mutations and two novel polymorphisms in the FBN1 gene". Hum Mutat. 2002,19:443-56. |