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One of PGC-1s primary roles is to stimulate mitochondrial biogenesis, the process by which new mitochondria are formed in the cell

For example, a 2017 study of 27 people with severe motor and intellectual disabilities found that carnitine levels were significantly lower in those who experienced constipation and that low levels were significantly related to constipation severity ( The study also showed that constipation severity decreased significantly after people received carnitine supplements of 4.522.5 milligrams per pound (mg/lb), or 1050 milligrams per kilogram (mg/kg), of body weight per day ( Summary A deficiency in carnitine is linked to constipation in those with severe intellectual and motor disabilities

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Patients with Mitochondrial Disease:There is an increased risk of valproate-induced acute liver failure and resultant deaths in patients with hereditary neurometabolic syndromes caused by DNA mutations of the mitochondrial DNA Polymerase (POLG) gene (e.g