BACE2 and Dravet syndrome: The final gene priorization list contains 8 genes located on HSA21 (supplementary material Table S5); ADAMTS1, ADAMTS5, APP, BACE2, PIGP, COL6A1, COL6A2 and COL18A1. In order to know which of them could explain the observable FBN1 deregulation in DS, we have tried to correlate the expression of those HSA21 genes with FBN1 in the meta-analysis and in DS heart study (note that co-expression with FBN1 comes from independent studies) finding that the top three genes with greater positive Pearson correlation and higher concordance between studies were found for BACE2, COL18A1 and COL6A2 (Table 2).