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Molecular Diagnosis of rare Mendelian autosomal diseases from Exome Sequencing data

Exome Sequencing represents a much more cost-effective way to study variations with respect to whole genome sequencing and currently constitutes the standard for pre-natal molecular diagnostics. In this study the aim was to identify the variants causing rare Mendelian autosomal diseases – frequency lower than 10−5 - in a set of ten children, having at disposal the exome sequencing data of the individuals and of the corresponding parents.

The analysis was performed based on the following assumptions:

  • Disease-causing variants are located on either chro- mosome 21 or chromosome 22,
  • Disease-causing variants affect only protein coding regions,
  • Alleles segregate independently,
  • A set of the possible diseases affecting the individuals, as well as their model of inheritance was given a priori,
  • Parental phenotype is unknown,
  • High-quality sequencing data is available – probabilistic modelling of SNPs is not necessary.

Variants were searched based on the criteria of rarity, correct allele segregation and deleteriousness using the online Ensembl Variant Effect Predictor (VEP) tool.

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