The UMD-FBN1 mutations database
Record ID: 170

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3422C>Tp.Pro1141LeuHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
CCGProCTGLeuC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #13 Yes, coding strandYes

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)
0.670.00 (pathogenous)71 (Probably pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA08PIT F0007 I01ProbandNANA?U.S.A

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
no clinical data

Reference


Reference IDPubMed IDReference
4910533071
Yuan B, Thomas JP, von Kodolitsch Y, Pyeritz RE. "Comparison of heteroduplex analysis, direct sequencing, and enzyme mismatch cleavage for detecting mutations in a large gene, FBN1". Hum Mutat 1999;14(5):440-6 .