ABSTRACT
Laboratory Animals ......................................................................... 193 9.2.4 Evidence from Humans .................................................................... 193
9.2.4.1 Menkes Disease ................................................................. 193 9.2.4.2 Wilson’s Disease ................................................................ 195
9.3 Selenium ....................................................................................................... 196 9.3.1 Human Selenium Metabolism .......................................................... 196 9.3.2 Role of Selenium in the Brain........................................................... 198 9.3.3 Evidence from Human Studies ......................................................... 198 9.3.4 Role of Selenium in the Developing Brain: Evidence from Humans ... 199
9.4 Conclusion .................................................................................................... 199 References ..............................................................................................................200
Copper is the third most abundant trace element in the body, after iron and zinc. It plays a number of essential biochemical roles, most notably as a cofactor for copper-dependent enzymes. Copper is essential for normal brain development and function and was rst recognized as essential in the mammalian brain in 1937 in sheep grazing in pastures with a low soil copper content. Lambs born to these ewes often died before birth, or had enzootic ataxia (swayback), manifested as reduced nerve myelination in the spinal cord, affecting motor coordination such that the lambs had difculty walking or were unable to stand. Lambs with this condition ultimately die of starvation, unless treated with copper supplements. Since the 1930s, numerous studies in laboratory animals and observations in humans with genetic abnormalities of copper metabolism have conrmed the essential role of copper in brain development and function. The pathology of the metabolic changes associated with copper deciency can be inuenced by the timing and duration of the deciency, and the degree of response can be different depending on the species (Uriu-Adams et al. 2010).
