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Heidelbaugh 2010

Conclusions Hyperammonaemia, frequently observed in the classical OAs (PA, MMA and IVA), is most often associated with metabolic decompensation in the neonatal period, and contributes to the risk of neurological damage

These effects arise because it does not act solely on the skins MC1R receptor, but also stimulates other melanocortin receptor subtypes located in central regions of the brain