Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.4179_4187del | p.Gly1394_Thr1396del | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
GAA | Glu | del9c | InF | In frame del | InF |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like #19 |
At the mRNA level | On restriction map |
No mechanism suspected | New restriction site(s): none Lost restriction site(s): none |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
AUS01NAD F0018 I79 | Proband | Male | de novo | first year of life (3 years old) | AUSTRALIA |
Phenotypic group | Disease |
NA | Neonatal MFS |
Symptom |
C-Mitral regurgitation |
C-Mitral regurgitation |
C-Mitral valve prolapse |
O-Ectopia lentis |
O-Iridodonesis |
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. |