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L-HCA is present in the brain although the concentrations were found to be about 500-fold lower than those of L-glu and 100-fold lower than those of L-asp in different areas of the rat brain (Kilpatrick and Mozley, 1986)

Furthermore, when we examined the expression of genes encoding enzymes that act as metabolic gatekeepers in CD4 + T cells, we found a BHD-induced upregulation of Cpt1 (fatty acid oxidation), Got1 (amino acid metabolism), Ndufs8 (OXPHOS), Sdha , Sdhb , Sdhc (TCA cycle) and Txnrd1 (redox balance) (Fig