The UMD-FBN1 mutations database
Record ID: 1913

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.7115G>Tp.Gly2372ValHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GGTGlyGTTValG->TTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP #07 conserved AA in TGFBPYes, non coding strandNo

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)
0.830.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA05HOU F0018 I0001ProbandMalefamilialU.S.A.

Phenotypic groupDisease
NATAAD

Clinical data


SymptomAge
C-Aortic insufficiency37
C-Asc. aortic dissection37

Reference


Reference IDPubMed IDReference
18019336958
Yamawaki T, Nagaoka K, Morishige K, Sadamatsu K, Tashiro H, Yasunaga H, Morisaki H, Morisaki T. "Familial thoracic aortic aneurysm and dissection associated with Marfan-related gene mutations: case report of a family with two gene mutations". Intern Med. 2009;48(7):555-8.