Unbiased gene expression analysis implicates the huntingtin polyglutamine tract in extra-mitochondrial energy metabolism.

More sensitive tests of groups of genes in functional pathways, using the Gene Ontology (GO) biological process ( Figure 4 A) and sigPathway gene set analysis [ 25 ] ( Figure 4 B), confirmed that the HD mutation and 3-NP were both associated with highly significant decreases in carbohydrate metabolism and, as reported previously [ 26 ], lipid (sterol/cholesterol) biosynthesis ( Figure 4 C).

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