Notably, the reduction of α-ketoglutarate dehydrogenase (KGDH) activity has recently been demonstrated in other neurodegenerative diseases, such as Alzheimer (Gibson et al., 1988), Parkinson (Mizuno et al., 1994, 1995), and Huntington diseases (Klivenyi et al., 2004), sharing neuropathological similarities with GCDH deficiency (Strauss and Morton, 2003). The gene discussed is GCDH; the disease is hyperinsulinemic hypoglycemia, familial, 4.