Nevertheless, hAAT significantly improved muscle action potential and histopathological features, and decreased circulating IL-6 in CMT1A mice; virtually all other parameters of disease severity showed a trend toward improvement.
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Alpha-1 Antitrypsin Reduces Disease Progression in a Mouse Model of Charcot-Marie-Tooth Type 1A: A Role for Decreased Inflammation and ADAM-17 Inhibition.
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