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These diseases are characterized by liver Cu accumulation without ATP7B mutation, though they do appear to have both a genetic and an environmental (i.e., high Cu in the diet) component [60,61,62]

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Compared with bacterial CGS, plant CGS have an N-terminal extension (amino acids 77-87) that displays a regulatory role ( Nicotiana tabacum transgenic plants highlighted both a strongly altered development and an impaired Met metabolism