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RNA interference and neuromuscular diseases: a focus on hereditary transthyretin amyloidosis

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Cys is the preferable amino acid substrate that is -glutamylated in the -GCS reaction (the Michaelis constant (Km) = 0.10.3 mM), but Cys-mimetic 2-aminobutyric acid (2AB) is also a good substrate [3]