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Human DLST wild-type allele is located in the vicinity of 14q24.3 and is approximately 22 kb in length. This allele, which encodes dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial protein, is involved in both lysine degradation and the tricarboxylic acid cycle.
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