Wild-type TREM2 carriers D11 (female, 48 years old was carrying ApoEɛ2/ɛ3, Tau H2/H2, TREM2C/C, and HLA-DR G/G) and D12 (male, 46 years old was carrying ApoEɛ3/ɛ3 Tau H2/H2, TREM2C/C, and HLA-DRA/G) had not developed dementia, indicating that H2 homozygosity could be protective in DS subjects (Table 2). The gene discussed is HLA-DRA; the disease is Dravet syndrome.