The UMD-FBN1 mutations database
Record ID: 226

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1302T>Gp.Tyr434XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TATTyrTAGStopT->GTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
Proline-rich Yes, coding strandNo

Mutation impact


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

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA03STA F0014 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.