The UMD-FBN1 mutations database
Record ID: 611

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3221G>Ap.Cys1074TyrHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGCCysTACTyrG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #12 Disulfide bonds 1074-1086 (C1)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)
0.830.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD05LON F0023 I01ProbandNANAU.K.

Phenotypic groupDisease
NANeonatal MFS

Clinical data


SymptomSeverity
C-Congestive heart failure
C-Mitral regurgitation
O-Cataract
O-Ectopia lentispossible
O-Glaucoma
S-Abnormal ears
S-Arachnodactyly (M)
S-Joint limitations

Reference


Reference IDPubMed IDReference
10912575662
Comeglio P, Evans AL, Brice GW, Anderlid BM, Child AH. "Gene symbol: FBN1. Disease: Marfan syndrome". Hum Genet. 2003 Jan;112(1):104.