Copper, Ceruloplasmin and Superoxide Dismutase (SOD) in Amyotrophic Lateral Sclerosis

Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis.

Copper-zinc superoxide dismutase (CuZnSOD, SOD1 protein) is an abundant copper- and zinc-containing protein that is present in the cytosol, nucleus, peroxisomes, and mitochondrial intermembrane space of human cells. Its primary function is to act as an antioxidant enzyme, lowering the steady-state concentration of superoxide, but when mutated, it can also cause disease. Over 100 different mutat...

Superoxide dismutase and amyotrophic lateral sclerosis.

Cover illustration: Lumbar spinal cord section from an ALS patient with sclerosis of the corticospinal tract. Klüver–Barerra staining. ABSTRACT Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons in the spinal cord, brain stem and motor cortex, leading to paralysis, respiratory failure and death. In about 5% of ALS cases, the disease is associa...

Superoxide Dismutase and Oxidative Stress in Amyotrophic Lateral Sclerosis

Oxidative stress is defined as the imbalance between reactive species such as free radicals and oxidants and the antioxidant defenses. Free radicals are molecules with one or more unpaired electrons, while oxidants are molecules with a high potential for taking electrons from other molecules. The more recognized reactive species are the reactive oxygen species (ROS), which include oxygen and it...

Mitochondrial Superoxide Dismutase Activity in Amyotrophic Lateral Sclerosis

Background: Evidence suggests that mitochondrial dysfunction and oxidative stress may be involved in the pathogenesis of Amyotrophic Lateral Sclerosis (ALS). Some studies show the presence of altered anti-oxidative defence enzyme activity in the blood of ALS patients. It has also been demonstrated that a superoxide-dismutase-1 (SOD1) enzyme fraction is located in the mitochondria. Objective: To...

Cu/Zn Superoxide Dismutase Misfolding in Amyotrophic Lateral Sclerosis