The UMD-FBN1 mutations database
Record ID: 3246

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.7861T>Gp.Ser2621AlaHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TCCSerGCCAlaT->GTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #42 Yes, coding strandNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
10.00 (pathogenous)65 (Probably pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
AUS01VIE F0012 I0001ProbandMaleNAAUSTRIA

Phenotypic groupDisease
NANA

Clinical data


SymptomAge
S-Characteristic facial appearance25
S-Scoliosis > 20° (M)(1)25

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]