Article
Increased outer arm and core fucose residues on the N-glycans of mutated alpha-1 antitrypsin protein from alpha-1 antitrypsin deficient individuals.
Journal of proteome research - 7 Feb 2014
McCarthy Cormac, Saldova Radka, O'Brien M Emmet, Bergin David A, Carroll Tomás P, Keenan Joanne, Meleady Paula, Henry Michael, Clynes Martin, Rudd Pauline M, Reeves Emer P, McElvaney Noel G
Abstract excerpt
Alpha-1 antitrypsin (AAT) is the major physiological inhibitor of a range of serine proteases, and in the lung, it maintains a protease-antiprotease balance. AAT deficiency (AATD) is an autosomal co-dominant condition with the Z mutation being the most common cause. Individuals homozygous for Z (PiZZ) have low levels of circulating mutant Z-AAT protein leading to premature emphysematous lung disease. Extensive...
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