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Figure 2 Information on pediatric studies are presented in section 8

Its primary role revolves around energy metabolism, specifically in the transport of fatty acids into the mitochondria, the powerhouse of the cell, where they undergo oxidation to produce energy in the form of adenosine triphosphate (ATP)

Of these articles, 155 were selected, summarized, and critically discussed to provide a comprehensive review

Organic acidaemias, including methylmalonic acidaemia, propionic acidaemia, isovaleric acidaemia and multiple carboxylase deficiency occur in ~1 in 21,000 births and typically lead to mild to moderate hyperammonaemia due to competitive inhibition of NAGS 5,6,7,8,9

Eine abwechslungsreiche, ausgewogene Ernhrung und eine gesunde Lebensweise sind von groer Bedeutung