The UMD-FBN1 mutations database
Record ID: 74

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1693C>Tp.Arg565XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CGAArgTGAStopC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #04 NoYes

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Nla III
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA07POR F0001 I03ProbandNANA?U.S.A

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
no clinical data
no clinical data

Reference


Reference IDPubMed IDReference
30-
Quan F, Sakai L, Popovich BW. "The identification of fibrillin mutations in Marfan syndrome using heteroduplex analysis and nucleotide sequencing". Am J Hum Genet 1995, 57 abstract 1936.