Mn-superoxide dismutase and pyruvate carboxylase are the two most important metalloenzymes of manganese. Evidence for the occurrence of overt manganese deficiency in human subjects has not been adequately described. Nonetheless, homeostasis of the metabolic synthesis of proteins, such as mucopolysaccharides and prothrombin, as well as carbohydrate and lipid (e.g., activates lipoprotein lipase activation, cholesterol, and sex hormone precursors) are important roles of manganese. Stores are found in the mitochondria of hepatic, renal, pancreatitic, bone, and skeletal muscle parenchyma. Deficiencies have been manifested as tardive dyski-nesia, epilepsy, diabetes mellitus, pancreatic insufficiency, and malnutrition. Deficiency has also been related to hair color changes, hypercholesterolemia, and prolonged prothrombin times. Manganese may accumulate in hepatically compromised patients.

Several experiences of manganese toxicity in individuals with cholestasis, including some confirmed by basal ganglia deposition as revealed by magnetic resonance imaging (MRI), were reported.87

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