The UMD-FBN1 mutations database
Record ID: 236

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3338A>Gp.Asp1113GlyHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GATAspGGTGlyA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #13 Ca2+ bindingYes, non coding strandNo

Mutation impact


At the mRNA levelOn restriction map
First nucleotide of the exon, cDNA not testedNew 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
USA03STA F0024 I01ProbandNANA?U.S.A

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
no clinical data

Reference


Reference IDPubMed IDReference
5410464652
Liu WO, Oefner PJ, Qian C, Odom RS, Francke U. "Denaturing HPLC-identified novel FBN1 mutations, polymorphisms, and sequence variants in Marfan syndrome and related connective tissue disorders". Genet Test 1997-98;1(4):237-42.