The UMD-FBN1 mutations database
Record ID: 704

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.7167_7168delCTp.Cys2390SerfsX15HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CTCLeudel2cFs.Stop at 2404Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP #07 conserved AA in TGFBP

Mutation impact


At the mRNA levelOn restriction map
Deletion of a repeated sequenceNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
AUS02WES F0018 I01ProbandNANA? (18 years old)AUSTRALIA

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Asc. aortic dilatation
C-Mitral valve prolapse
S-Arachnodactyly (M)
S-Characteristic facial appearance
S-Crowding teeth (m)
S-High arched palate
S-Joint hypermobility (m)
SI-Significant striae atrophicae (m)(1)

Reference


Reference IDPubMed IDReference
11414695540
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.