When used for the long-term treatment of organic acidurias, carglumic acid decreased/normalized ammonia levels, increased protein intake, increased bodyweight and/or improved bone mineral density (Table 5) [30,31,32]
Downregulation of SOD1 in vitro and in vivo models has been associated with neuronal death [61], while overexpression of SOD1 in transgenic mice has been associated with protection of the cerebral tissue in several pathological conditions such as ischemia or Parkinsons disease Lowered SOD activity has been linked to a high risk of oxidative stress, which can lead to a number of diseases, ranging including diabetes, heart failure, stroke, hypertension, high cholesterol, and atherosclerosis [14,62]
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