The UMD-FBN1 mutations database
Record ID: 262

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.8057G>Tp.Cys2686PheHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TGTCysTTTPheG->TTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #43 Disulfide bonds 2674-2686 (C6)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)
10.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA03STA F0049 I05ProbandNANA? (27 years old)U.S.A

Phenotypic groupDisease
Type IV dClassical MFS

Clinical data


Symptom
C-Aortic surgery
C-Asc. aortic dilatation
O-Myopia
S-Arachnodactyly (M)
S-High arched palate
S-Joint limitations
S-Pectus carinatum (M)(2)
SI-Significant striae atrophicae (m)(1)

Reference


Reference IDPubMed IDReference
5410464652
Liu WO, Oefner PJ, Qian C, Odom RS, Francke U. "Denaturing HPLC-identified novel FBN1 mutations, polymorphisms, and sequence variants in Marfan syndrome and related connective tissue disorders". Genet Test 1997-98;1(4):237-42.