The UMD-FBN1 mutations database
Record ID: 1181

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.[3203C>G; 3204A>G]p.Pro1068ArgHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGCCysCGGArgT->C/C->GTs/Tv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #11 Disulfide bonds 1055-1068 (C6)NoNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): Hpa II, Msp I, Nla IV
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.830.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
AUS02WES F0062 I01ProbandFemalede novoAUSTRALIA

Phenotypic groupDisease
NANeonatal MFS

Clinical data


Symptom
C-Aortic insufficiency
C-Asc. aortic dilatation
CF-Broad nasal bridge
CF-Deep set eyes
O-Megalocornea
O-Trabeculodysgenesis
S-Abnormal ears
S-Arachnodactyly (M)
S-Joint limitations
SI-Loose, redundant skin

Reference


Reference IDPubMed IDReference
13915264290
Whitelaw CM, Anwar S, Ades LC, Gole GA, Elder JE, Savarirayan R. "Primary trabeculodysgenesis in association with neonatal Marfan syndrome". Am J Med Genet A. 2004 Aug 1;128(4):418-21.