The UMD-FBN1 mutations database
Record ID: 1126

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.7988G>Cp.Cys2663SerHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGTCysTCTSerG->CTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #43 Disulfide bonds 2652-2663 (C3)NoNo

Mutation impact


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

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
10.48 (non pathogenous)71 (Probably pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
JAP06HIR F0003 I0001ProbandNAfamilial41 years oldJAPAN

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Asc. aortic dissection
C-Mitral regurgitation
C-Mitral valve prolapse
S-Arachnodactyly (M)
S-Pectus excavatum moderate (m)(1)
SI-Significant striae atrophicae (m)(1)

Reference


Reference IDPubMed IDReference
11915221638
Uyeda T, Takahashi T, Eto S, Sato T, Xu G, Kanezaki R, Toki T, Yonesaka S, Ito E. "Three novel mutations of the fibrillin-1 gene and ten single nucleotide polymorphisms of the fibrillin-3 gene in Marfan syndrome patients". J Hum Genet. 2004;49(8):404-7.