Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.4485delC | p.Thr1496ArgfsX24 | Heterozygous | Mutation |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
ACC | Thr | del1c | Fs. | Stop at 1519 | Fr. |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
cb EGF-like #22 |
At the mRNA level | On restriction map |
Deletion in a stretch of nucleotides | New restriction site(s): none Lost restriction site(s): none |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
UKD04OXF F0004 I04 | Proband | NA | familial | ? (31 years old) | U.K. |
Phenotypic group | Disease |
NA | Classical MFS + |
Symptom |
C-Asc. aortic dilatation |
C-Mitral valve prolapse |
S-Arachnodactyly (M) |
S-Arm span/height >1.05 (M) |
S-Chest deformity (unspecified) |
S-High arched palate |
S-Scoliosis > 20° (M)(1) |
SI-Significant striae atrophicae (m)(1) |
Reference ID | PubMed ID | Reference |
58 | 10647894 | Halliday D, Hutchinson S, Kettle S, Firth H, Wordsworth P, Handford PA. "Molecular analysis of eight mutations in FBN1". Hum Genet 1999 Dec;105(6):587-97. |