The UMD-FBN1 mutations database
Record ID: 613

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.491A>Gp.Asn164SerHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AATAsnAGTSerA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
EGF-like#03 Yes, non coding strandNo

Mutation impact


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

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.830.00 (pathogenous)53 (Probable polymorphism)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD05LON F0015 I01ProbandFemalefamilialU.K.

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


Symptom
O-Ectopia lentis
S-Reduced US/LS ratio <0.87 (M)
SI-Significant striae atrophicae (m)(1)

Reference


Reference IDPubMed IDReference
10812446365
Comeglio P, Evans AL, Brice G, Cooling RJ, Child AH. "Identification of FBN1 gene mutations in patients with ectopia lentis and marfanoid habitus". Br J Ophthalmol. 2002 Dec;86(12):1359-62.