The UMD-FBN1 mutations database
Record ID: 604

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.5723C>Tp.Thr1908IleHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ACAThrATAIleC->TTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #28 Ca2+ bindingNoNo

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)94 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
GER03HAN F0016 I10ProbandMaleNA10 years oldGERMANY

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


Symptom
C-Mitral regurgitation
C-Mitral valve prolapse
S-High arched palate
S-Plain pes planus (M)(1)

Reference


Reference IDPubMed IDReference
10712402346
Rommel K, Karck M, Haverich A, Schmidtke J, Arslan-Kirchner M. "Mutation screening of the fibrillin-1 (FBN1) gene in 76 unrelated patients with Marfan syndrome or Marfanoid features leads to the identification of 11 novel and three previously reported mutations". Hum Mutat. 2002 Nov;20(5):406-7.