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However, excess glutamine in a protein is of pathological importance, and a number of neurodegenerative diseases have been found to be due to a CAG expansion that causes expansion of glutamine repeats in affected proteins (CAA and CAG codons are responsible for the insertion of glutamine from its transfer RNA with its anti-codon triplet into the genetically determined position of the coded polypeptide chain)

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Saccharopine is further converted to a-aminoadipate semialdehyde in a reaction catalyzed by the saccharopine dehydrogenase domain ( Figure 4 )