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Research studies, conducted in several in vitro and in vivo animal and clinical models have shown that these agents are active in treatment of abnormal cellular metabolism induced by mitochondrial inhibitors, or those caused by L-carnitine deficiency or insufficiency created by iatrogenic factors (e.g., valproate, pivalic acid)

Appl Sci 11(18):8522 Paulson HL, Shakkottai VG, Clark HB, Orr HT (2017) Polyglutamine spinocerebellar ataxias - from genes to potential treatments