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Palmitoylcarnitine regulates estrification of lipids and promotes palmitoylation of GAP-43

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Mitochondrial homeostasis Carnitine pool homeostasis plays an important role in maintaining mitochondrial function and integrity [12, 13]

Conditions for which this effect has been demonstrated are: glutaric aciduria II, methyl malonic aciduria, propionic acidemia, and medium chain fatty acylCoA dehydrogenase deficiency.7,8 Autointoxication occurs in these patients due to the accumulation of acylCoA compounds that disrupt intermediary metabolism