The UMD-FBN1 mutations database
Record ID: 2042

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3989dupp.His1331ThrfsX19HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GCAAlains1cFs.Stop at 1349Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #18 

Mutation impact


At the mRNA levelOn restriction map
Duplication flanked by direct repeatsNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA15BOS F0001 I0001ProbandMalefamilialU.S.A.

Phenotypic groupDisease
NAClassical MFS +

Clinical data


SymptomSeverity
C-Cardiac malformationAtrial septal defect
S-Abnormal ears
S-Arachnodactyly (M)
SI-Other herniae

Reference


Reference IDPubMed IDReference
18620012798
Chiu CH, Thakuria J, Agrawal PB. "Novel CHD7 and FBN1 mutations in an infant with multiple congenital anamolies". Indian J Pediatr. 2010 Feb;77(2):208-9.