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• W2114584987 abstract "Frontotemporal lobar degeneration with motor neuron disease (FTLD-MND) is characterized by neuronal cytoplasmic inclusions containing TDP-43. Apolipoprotein E4 (apoE4), derived from the apoE ϵ4 allele, enhances brain atrophy in FTLD through unknown mechanisms. Here, we studied two siblings with C9ORF72-linked familial FTLD-MND, an apoE ϵ4 homozygote and an apoE ϵ3 homozygote. The apoE ϵ4 homozygote had more cognitive-behavioral symptoms, fronto-insulo-temporal atrophy, and apoE fragments and aggregates in the anterior cingulate cortex. ApoE formed complexes with TDP-43 that were more abundant in the apoE ϵ4 homozygote. Although differences seen in a sibling pair could arise due to chance, these findings raise the possibility that apoE4 exacerbates brain pathology in FTLD through formation of neurotoxic apoE fragments and interactions with TDP-43." @default.
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• W2114584987 date "2013-06-01" @default.
• W2114584987 modified "2023-10-03" @default.
• W2114584987 title "ApoE and TDP-43 neuropathology in two siblings with familial FTLD-motor neuron disease" @default.
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• W2114584987 doi "https://doi.org/10.1080/13554794.2012.667124" @default.
• W2114584987 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3655113" @default.
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