High-throughput targeted lipidomic data obtained from biological replicates (n=12) of four cellular models of Alexander disease using U-87 MG non-transfected and GFP-GFAP expressing cells (WT, R239C, R239G, R79C, and E373K variants).
Alexander disease (AxD) is a rare, severe neurodegenerative disorder caused by mutations in the glial fibrillary acidic protein (GFAP). While the exact disease mechanism remains unknown, existing studies suggest that the mutant GFAP influences many cellular processes, including cytoskeleton stabilit...
In this work we used our zebrafish model of Alexander Desease (AxD), aiming at unraveling the main pathways involved in AxD pathogenesis performing the first multi-omics analysis on a in vivo model of AxD. The obtained results have been functionally validated and they open the way to further investi...
Alexander disease (AxD) is a fatal neurodegenerative disorder characterized by the presence in astrocytes of protein aggregates called Rosenthal fibers (RFs). In this work, we used a new biochemical fractionation method to enrich for RFs followed by analysis of this fraction using quantitative iTRA...
ORGANISM(S): Mus musculus (Mouse) Homo sapiens (Human)
We performed label-free micro-data-independent acquisition (µDIA) to assay brain proteins from a severe model of Alexander disease (AxD, lethal at postnatal day ~35). Of the 5,005 proteins quantified, we observed upregulation of adipocytokine signaling, PPAR, insulin resistance, and glutathione pat...
Alzheimer’s disease (AD) is characterized by a prolonged asymptomatic phase before cognitive decline emerges, yet the mechanisms driving symptom onset remain unclear. Here, we hypothesized that the transition from asymptomatic to symptomatic disease is linked to dysfunction of brain–immune communica...
