The UMD-FBN1 mutations database
Record ID: 335

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3058A>Gp.Thr1020AlaHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
ACAThrGCAAlaA->GTs

StructureKey Residue (HCD)Pyrimidin doubletCpG
 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)
0.830.66 (non pathogenous)71 (Probably pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
GER01BER F0005 I47ProbandFemalefamilialat 4 years oldGERMANY

Phenotypic groupDisease
NAClassical MFS

Clinical data


SymptomSeverity
S-Arachnodactyly (M)borderline
S-High arched palate
S-Increased body length
S-Joint hypermobility (m)

Reference


Reference IDPubMed IDReference
8011175294
Tiecke F, Katzke S, Booms P, Robinson P, Neumann L, Godfrey M, Mathews KR, Scheuner M, Hinkel GK, Brenner RE, H*vels-GŸrich H, Hagemeier C, Fuchs J, Skovby F, Rosenberg T. "Classic , early-onset severe and neonatal Marfan syndrome : Thirteen mutations and genotype-phenotype correlations in FBN1 exons 24-40". Eur J Hum Genet 2001, 9:13-21.