The UMD-FBN1 mutations database
Record ID: 3318

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3713_3714delinsCAp.Asp1238HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ATCIleindelsindelsindelsindels

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #16 Ca2+ bindingNoNo

Mutation impact


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

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.83 - 65 (Probably pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD09SAL F0018 I0001ProbandNANAU.K.

Phenotypic groupDisease
NAMFS

Clinical data


Symptom

Reference


Reference IDPubMed IDReference
24221895641
Robinson DO, Lin F, Lyon M, Raponi M, Cross E, White HE, Cox H, Clayton-Smith J, Baralle D. "Systematic screening of FBN1 gene unclassified missense variants for splice abnormalities". Clin Genet. 2012 Sep;82(3):223-31.