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Martin TA, Harrison G, Mansel RE, Jiang WG

2019;2: e00051

Children should start at ~1/4 of adult (2 pumps per day) dosage and work up

Adenosylcobalamin (energy production) works well with patients with low energy, often given in combination with hydroxocobalamin

Finally, it is also notable, in light of the reduction in mitochondrial subunit expression seen in the downregulated dataset, that multiple respirasome proteins were also found to be upregulated, including electron transfer flavoprotein-ubiquinone oxidoreductase, which is responsible for linking fatty acid and amino acid degradation with oxidative phosphorylation and was increased 1.3-fold (p=0.023)