The UMD-FBN1 mutations database
Record ID: 148

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.5162G>Ap.Cys1721TyrHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGTCysTATTyrG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP#05 C in disulfide bonds 1721-1748NoNo

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)
10.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD03DUN F0006 I01ProbandFemalefamilial? (57 years old)U.K.

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Asc. aortic dissection
O-Myopia
S-Arachnodactyly (M)
S-Chest deformity (unspecified)
S-Dolichostenomelia
S-High arched palate
SI-Significant striae atrophicae (m)(1)

Reference


Reference IDPubMed IDReference
70-
Black C, Boxer M (Personnal communication 2000).