The UMD-FBN1 mutations database
Record ID: 3244

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.6947delGp.Cys2316LeufsX82HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGTCysdel1bFs.Stop at 2397Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like#36 Disulfide bonds 2302-2316 (C4)

Mutation impact


At the mRNA levelOn restriction map
No mechanism suspectedNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
AUS01VIE F0010 I0001ProbandMalefamilialAUSTRIA

Phenotypic groupDisease
NANA

Clinical data


SymptomAge
S-Arachnodactyly (M)8
S-Plain pes planus (M)(1)8

Reference


Reference IDPubMed IDReference
27124199744
Pees C, Michel-Behnke I, Hagl M, Laccone F. "Detection of 15 novel mutations in 52 children from 40 families with the Marfan or Loeys Dietz syndrome and phenotype-genotype correlations". Clin Genet. 2013 Nov 6. doi: 10.1111/cge.12314. [Epub ahead of print]