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Since these two reactions are catalyzed by the bifunctional enzyme, AASS, defects can be found in either the N-terminal lysine:2-oxoglutarate reductase activity or the C-terminal saccharopine dehydrogenase activity

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In such cases, targeted radiological imaging, such as magnetic resonance imaging (MRI) 49 or computed tomography (CT), 50 is essential to identify underlying structural or vascular causes

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