The UMD-FBN1 mutations database
Record ID: 685

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3110_3122delp.Ser1037ThrfsX47HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AGCSerdel13bFs.Stop at 1083Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #11 

Mutation impact


At the mRNA levelOn restriction map
Deletion flanked by direct repeatsNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
CHI02CHA F0001 I0001ProbandMaleNA13 years oldCHINA

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Mitral regurgitation
O-Ectopia lentis
S-Arachnodactyly (M)
S-Increased body length
S-Scoliosis > 20° (M)(1)

Reference


Reference IDPubMed IDReference
11212890380
Wang B, Hu D, Xia J, Li Q, Yang J, Lu G. "FBN1 mutation in Chinese patients with Marfan syndrome and its gene diagnosis using haplotype linkage analysis". Chin Med J (Engl). 2003 Jul;116(7):1043-6.