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The breakdown process of BCAAs is similar in all species, initially forming branched-chain -keto acids (BCKAs) via branched-chain amino acid transferase (BCATs) and transferring nitrogen to nitrogen receptors (the most common nitrogen receptor is -ketoglutaric acid (-KG) to form glutamate)

In Angelman Syndrome, a neurodevelopmental disorder characterized by developmental delay, intellectual disability, seizures, and motor dysfunction, the UBE3A gene is typically inactivated in neurons through imprinting (Buiting et al., 2016

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