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Carnitine availability limits the rate of this pathway, known as the Lands cycle, which is activated in response to oxidant stress (e.g., in response to exercise [109] or in patients with hemoglobinopathies, such as sickle cell trait [110] and sickle cell disease [111,112])

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However, this precise, finely tuned system is now increasingly subject to impairment by environmental factors, including endocrine disruptors and generators of oxidative stress in general [47,48,49,50,51,52,53,54,55,56]

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Mutations in SOD1 can cause familial ALS (several pieces of evidence also show that wild-type SOD1, under conditions of cellular stress, is implicated in a significant fraction of sporadic ALS cases, which represent 90% of ALS patients.), [58] by a mechanism that is presently not understood, but not due to loss of enzymatic activity or a decrease in the conformational stability of the SOD1 protein