The UMD-FBN1 mutations database
Record ID: 230

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.2399delCp.Pro800LeufsX3HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CCTProdel1bFs.Stop at 802Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #08 

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
USA03STA F0018 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.