The UMD-FBN1 mutations database
Record ID: 467

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.5791_5793delGTTp.Val1931delHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GTTValdel3aInFIn frame delInF

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #29 Ca2+ binding

Mutation impact


At the mRNA levelOn restriction map
Deletion flanked by direct repeatsNew restriction site(s): none
Lost restriction site(s): none

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
GER01BER F0046 I52ProbandFemalefamilialUnknownGERMANY

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
C-Mitral regurgitation
C-Mitral valve prolapse
CF-Dolichocephaly
O-Myopia
S-Arachnodactyly (M)
S-Characteristic facial appearance
S-Crowding teeth (m)
S-High arched palate
S-Increased body length
S-Joint hypermobility (m)
S-Scoliosis > 20° (M)(1)

Reference


Reference IDPubMed IDReference
9512203992
Katzke S, Booms P, Tiecke F, Palz M, Pletschacher A, Turkmen S, Neumann LM, Pregla R, Leitner C, Schramm C, Lorenz P, Hagemeier C, Fuchs J, Skovby F, Rosenberg T, Robinson PN. "TGGE screening of the entire FBN1 coding sequence in 126 individuals with marfan syndrome and related fibrillinopathies". Hum Mutat. 2002 Sep;20(3):197-208.