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The disease is caused by the amplification of the CAG trinucleotide repeat in the first exon of the Huntingtin gene on chromosome 4 (Stoker et al., 2022)

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In nature, ALA is found only in the R form (Figure 1) and is present in the body both in free form and conjugated to conserved lysine residues in an amide bond, thus making this isomer a key cofactor in different biological systems [7]

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