The UMD-FBN1 mutations database
Record ID: 1165

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1753G>Cp.Gly585ArgHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GGAGlyCGAArgG->CTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #05 conserved AA in cbEGF-likeYes, non coding strandNo

Mutation impact


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

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

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
FRA03MON F0021 I0001ProbandFemalefamilialFRANCE

Phenotypic groupDisease
NAClassical MFS

Clinical data


SymptomSeverityAge
O-Ectopia lentissurgery29
S-Arachnodactyly (M)29
S-Protusio acetabulæ (M)(2)29
SI-Significant striae atrophicae (m)(1)29

Reference


Reference IDPubMed IDReference
19119802897
Khau Van Kien P, Baux D, Pallares-Ruiz N, Baudoin C, Plancke A, Chassaing N, Collignon P, Drouin-Garraud V, Hovnanian A, Martin-Coignard D, Collod-B*roud G, B*roud C, Roux AF, Claustres M. "Missense mutations of conserved glycine residues in fibrillin-1 highlight a potential subtype of cb-EGF-like domains". Hum Mutat. 2010 Jan;31(1):E1021-42.