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J Neurochem 97:424434 Miecz D, Januszewicz E, Czeredys M, Hinton BT, Berezowski V, Cecchelli R, Nalecz KA (2008) Localization of organic cation/carnitine transporter (OCTN2) in cells forming the bloodbrain barrier

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

Figure 2 Information on pediatric studies are presented in section 8

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