The UMD-FBN1 mutations database
Record ID: 3210

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3959G>Ap.Cys1320TyrHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGTCysTATTyrG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #17 Disulfide bonds 1307-1320 (C6)NoNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): Csp6 I, Rsa I

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
USA21MIL F0001 I0002RelativeMalefamilialU.S.A.

Phenotypic groupDisease
NAProbable MFS

Clinical data


Symptom
S-Abnormal ears
S-Characteristic facial appearance

Reference


Reference IDPubMed IDReference
25723930893
Elshershari H, Harris C. "Paternal fibrillin-1 mutation transmitted to an affected son with neonatal marfan syndrome: the importance of early recognition". Cardiol Young. 2013 Aug 9:1-4.