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Description

M.KoningA.KuhnleG

AD patients exhibit fewer, dysfunctional mitochondria and downregulated OXPHOS genes, leaving neurons hypometabolic

10.1186/s13063-024-08584-x 197

The disorder usually presents in early childhood and is characterized by episodes of hypoketotic hypoglycemia (that can cause encephalopathy), hepatomegaly, elevated liver enzymes (transaminases), and hypoammonemia in infants