The UMD-FBN1 mutations database
Record ID: 1953

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1092_1094dupp.Cys365XHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGCCysins3cInFStop at 365Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP#01 C in disulfide bonds 345-365

Mutation impact


At the mRNA levelOn restriction map
Duplication of a repeated sequenceNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
GER03HAN F0065 I0005ProbandNANAGERMANY

Phenotypic groupDisease
NANA

Clinical data


Symptom
Clinical data will be implemented as soon as possible

Reference


Reference IDPubMed IDReference
18219159394
S*ylen B, Singh KK, Abuzainin A, Rommel K, Becker H, Arslan-Kirchner M, Schmidtke J. "Prevalence of dural ectasia in 63 gene-mutation-positive patients with features of Marfan syndrome type 1 and Loeys-Dietz syndrome and report of 22 novel FBN1 mutations". Clin Genet. 2009 Mar;75(3):265-70.