The UMD-FBN1 mutations database
Record ID: 549

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.976delAp.Thr326ProfsX4HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ACCThrdel1aFs.Stop at 329Fr.

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #02 

Mutation impact


At the mRNA levelOn restriction map
No mechanism suspectedNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA04NOR F0012 I3524ProbandFemalefamilialUnknownU.S.A

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Aortic insufficiency
C-Asc. aortic dilatation
O-Myopia
S-Scoliosis > 20° (M)(1)

Reference


Reference IDPubMed IDReference
102-
Hyland JC (Personal communication 2003).