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Following the initial dehydrogenation, the resulting long-chain enoyl-CoAs undergo hydration, a second dehydrogenation step, and finally thiolytic cleavage catalyzed by mitochondrial trifunctional protein (MTP) [10]
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Where clinically appropriate and safe, alternative agents with more weight-neutral profiles may be considered
Thus, Htt reduces Cu 2+ to Cu 1+ , and copper promotes its aggregation, mediated by histidine residues ( Figure 6 schematizes the pathways that lead to symptoms of Huntingtons disease as a consequence of misfolded mutant Htt
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