The UMD-FBN1 mutations database
Record ID: 3129

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.4753T>Ap.Tyr1585AsnHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
TACTyrAACAsnT->ATv

StructureKey Residue (HCD)Pyrimidin doubletCpG
TGFBP#04 conserved AA in TGFBPNoNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): Csp6 I, Rsa I

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
GER03HAN F0107 I0001ProbandFemaleNAGERMANY

Phenotypic groupDisease
NANA

Clinical data


SymptomAge
C-Mitral valve prolapse27

Reference


Reference IDPubMed IDReference
24321883168
Sheikhzadeh S, Kade C, Keyser B, Stuhrmann M, Arslan-Kirchner M, Rybczynski M, Bernhardt AM, Habermann CR, Hillebrand M, Mir T, Robinson PN, Berger J, Detter C, Blankenberg S, Schmidtke J, von Kodolitsch Y. "Analysis of phenotype and genotype information for the diagnosis of Marfan syndrome". Clin Genet. 2012 Sep;82(3):240-7.