The UMD-FBN1 mutations database
Record ID: 690

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.510C>Gp.Tyr170XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TACTyrTAGStopC->GTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
EGF-like#03 NoNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Dde I
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
AUS02WES F0004 I01ProbandNANA? (10 years old)AUSTRALIA

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Asc. aortic dilatation
C-Mitral valve prolapse
CNS-Lumbosacral dural ectasia
S-Arachnodactyly (M)
S-Characteristic facial appearance
S-Crowding teeth (m)
S-High arched palate
S-Joint hypermobility (m)
S-Plain pes planus (M)(1)
S-Reduced US/LS ratio <0.87 (M)

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.