The UMD-FBN1 mutations database
Record ID: 3361

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1061C>Gp.Thr354SerHeterozygousPolymorphism?

wt codonwt aamutant codonmutant aamutational eventmutation type
ACCThrAGCSerC->GTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP#01 conserved AA in TGFBPYes, coding strandNo

Mutation impact


At the mRNA levelOn restriction map
Not tested on cDNA (needed)New restriction site(s): none
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.830.79 (non pathogenous)53 (Probable polymorphism)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA22DAL F0003 I0001ProbandFemalefamilialU.S.A.

Phenotypic groupDisease
NAEhlers-Danlos Syndrome

Clinical data


SymptomAge
O-Myopia7
S-Joint hypermobility (m)31
SI-Easy bruising7
SI-Significant striae atrophicae (m)(1)7
SI-Soft skin31
SI-Translucent skin31

Reference


Reference IDPubMed IDReference
28524664531
Wilson GN. "Exome analysis of connective tissue dysplasia: Death and rebirth of clinical genetics?" Am J Med Genet A. 2014 May;164(5):1209-12.