The UMD-FBN1 mutations database
Record ID: 116

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.4828T>Gp.Cys1610GlyHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGCCysGGCGlyT->GTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #23 Disulfide bonds 1610-1622 (C1)NoNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Hae III, Sau96 I
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
10.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD01EDI F0011 I01ProbandFemalede novo? (29 years old)U.K.

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Aortic insufficiency
O-Myopia
O-Retinal detachment
S-Arachnodactyly (M)
S-Characteristic facial appearance
S-Chest deformity (unspecified)
S-Joint hypermobility (m)
S-Joint limitations

Reference


Reference IDPubMed IDReference
419338581
Hayward C, Porteous ME, Brock DJ. "Mutation screening of all 65 exons of the fibrillin-1 gene in 60 patients with Marfan syndrome: report of 12 novel mutations". Hum Mutat 1997;10(4):280-9.