Reactive centre loop mutants of α-1-antitrypsin reveal position-specific effects on intermediate formation along the polymerization pathway.

Substitutions at P 14 P 12 , P 6 P 4 and P 4 led to a marked decrease in polymer levels, but the reduction seen with the P 10 P 8 variant did not reach statistical significance, and for the P 6 and P 2 P 1′ loop mutants, the intracellular polymer burden was equivalent to the Z α 1 -antitrypsin control.

also in 111,027 other papers