References
- Papers of special note have been highlighted as:
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•• The documentation of replacement therapy with AAT as a logical approach to long-term medical treatment in patients with AATD
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• Demonstrates that TNF-α release by alveolar macrophages is modulated by AAT
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•• Demonstrates that mice exposed to cigarette smoke and treated with exogenous AAT were protected against the development of emphysema and increased levels of serum TNF-α
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•• The identification of misfolded Z-AAT protein within the ER of neutrophils leading to the release of TNF-α
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• AAT modulates protein phosphatase 2A to modulate inflammatory induced by TNF signaling in the lung
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•• Documentation of the ability of inhaled AAT to decreased the levels of elastase activity, neutrophils and pro-inflammatory cytokines in airways of patients with CF
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•• Evidence that purified AAT augmentation slows progression of emphysema
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