The UMD-FBN1 mutations database
Record ID: 1889

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3602G>Ap.Cys1201TyrHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGCCysTACTyrG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #15 Disulfide bonds 1201-1212 (C1)NoNo

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)
0.670.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
TUR02ANK F0001 I0001ProbandMalede novoat birth22 daysTURKEY

Phenotypic groupDisease
NANeonatal MFS

Clinical data


Symptom
C-Mitral regurgitation
C-Mitral valve prolapse
C-Pulmonary valve insufficiency
C-Tricuspid valve prolapse
CF-Down-slanting palpebral fissures
CF-Micrognathia
S-Abnormal ears
S-Arachnodactyly (M)
S-High arched palate
S-Joint limitations
S-Muscular hypotonia
SI-Loose, redundant skin

Reference


Reference IDPubMed IDReference
17118388785
Derbent M, Anuk D, Tarcan A, Varan B, Gurakan B, Tokel K. "Functional pulmonary atresia in a patient with neonatal Marfan syndrome caused by a c.3602G>A mutation in exon 29 of the FBN1 gene". Clin Dysmorphol. 2008 Apr;17(2):127-8.