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The cell-type specific transcriptional profiling of mutant SOD1 mice shows early abnormalities in two nuclear receptors, the peroxisome proliferator-activated receptor and liver X receptor, which regulate mitochondrial biogenesis, oxidative metabolism, and mitochondrial fatty acid metabolism (126)

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Modulatory Profiling of Small Molecule Inhibitors The following small molecule inhibitors were tested in a ten-point, four-fold dilution series for their ability to prevent erastin-induced death in HT-1080, Calu-1 and BJ-eLR cells (high dose of dilution series in brackets): carbobenzyloxy-valyl-alanyl-aspartyl-[O-methyl]-fluoromethylketone (z-VAD-fmk, 184 M), necrostatin-1 (Nec-1, 19.3 M), cyclosporine A (CspA, 33.2 M), N-Acetyl-Leu-Leu-Nle-CHO (ALLN, 40 M), (2S,3S)-trans-epoxysuccinyl-L-leucylamido-3-methylbutane ethyl ester (E64d, 400 M), bafilomycin A1 (Baf A1, 4 M), 3-methyladenine (3-MA, 6.25 mM), chloroquine (Chq, 250 M), deferoxamine (DFO, 400), 6-hydroxy-2,5,7,8-tetramethylchroman-2-carboxylic acid (trolox, 320 M), 1,4-diamino-2,3-dicyano-1,4-bis (2-aminophenylthio)butadiene (U0126, 52.4 M) and cycloheximide (5.6 M)