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This idea is supported by other neurodegenerative diseases experiencing severe localized copper deficiency, such as the motor neurons of ALS patients (Hilton et al., 2024), PD patients with up to 50% reduction of copper seen in the substantia nigra (Bisaglia and Bubacco, 2020), brain copper reductions in prion diseases (Sorenson, 2001), and notably Menkes disease which is characterized by an X-linked defect in copper transporting ATP7-A gene that leads to severe systemic copper deficiency and progressive neurodegeneration (Tmer and Mller, 2010)

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It was suggested that a functional B 12 deficiency is present in these patients, despite normal B 12 serum levels

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