The UMD-FBN1 mutations database
Record ID: 349

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS22+1G>A (c.2728+1G>A)HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GATAspspl+1Spl.G->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #10 Ca2+ binding

Mutation impact


At the mRNA levelOn restriction map
Not tested on cDNANew restriction site(s): none
Lost restriction site(s): none

Impact on splicing
Splice site type Wild type sequence CV Mutant type sequence CV Variation (%)
Donor
AAGgtattt
77.9 _
AAGatattt
51.1 _ *
-34.4 %

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
UKD03DUN F0013 I01ProbandNANA?U.K.

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
no clinical data

Reference


Reference IDPubMed IDReference
84-
Barber R, Boxer M (Personal communication 2001).