The UMD-FBN1 mutations database
Record ID: 1894

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.5339G>Ap.Gly1780GluHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GGAGlyGAAGluG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #25 conserved AA in cbEGF-likeYes, non coding strandNo

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
FRA03MON F0405 I0001ProbandFemalefamilialFRANCE

Phenotypic groupDisease
NAClassical MFS

Clinical data


SymptomAge
O-Ectopia lentis12
S-Arachnodactyly (M)12
S-Crowding teeth (m)12
S-High arched palate12
S-Joint hypermobility (m)12

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.