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N-acetyl cysteine, or NAC, is a stable form of the amino acid L-cysteine

Vitamin B6 and pantothenic acid (also referred to as vitamin B5) contribute to normal energy yielding-metabolism and to the reduction of tiredness and fatigue

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Statistical tests comprised unpaired two-tailed Student's t-test and one-way or two-way analysis of variance (ANOVA), as appropriate, followed by Bonferroni post hoc tests

While we could demonstrate evidence of L-carnitine deficiency and accumulation of -BB as direct consequences of gbh-1/BBOX1 deficiency, the absence of significant changes in other metabolites confirms that the loss of GBH-1 selectively impacts the L-carnitine biosynthesis pathway without significantly affecting other metabolic pathways in amino acid, nucleotide, and energy metabolism that are not dependent on L-carnitine (Fig