The UMD-FBN1 mutations database
Record ID: 2039

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3863delCp.Pro1288GlnfsX5HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CCAProdel1bFs.Stop at 1292Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #17 conserved AA in cbEGF-like

Mutation impact


At the mRNA levelOn restriction map
Deletion in a stretch of nucleotidesNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
NET04NIJ F0003 I0001ProbandFemalefamilial5 years oldNETHERLAND

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom

Reference


Reference IDPubMed IDReference
18419059503
Van Dijk FS, Hamel BC, Hilhorst-Hofstee Y, Mulder BJ, Timmermans J, Pals G, Cobben JM. "Compound-heterozygous Marfan syndrome". Eur J Med Genet. 2009 Jan-Feb;52(1):1-5.