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Homozygous missense mutation of NDUFV1 as the cause of infantile bilateral striatal necrosis

Mitochondrial function assay in this study showed that OD 550nm values were remarkably up-regulated from the NC (0.38 0.04), GSH (0.43 0.07), SeNPs (0.59 0.04) groups to the GSH + SeNPs group (0.68 0.05

The treatment of a severe deficiency is divided into two phases: High-dose restorative therapy (injection) Through injections with B12, the bodys depleted stores are replenished and blood values quickly raised Maintenance treatment (tablets/capsules or injection) Through an oral follow-up treatment, values are stabilised and maintained

2013;17(Suppl 1):S367-S373

doi: 10.1002/hep.1840150631