The reactive site of alpha 1-antitrypsin is C-terminal, not N-terminal.

alpha 1-Antitrypsin recovered from trypsin-alpha 1-antitrypsin complexes was shown to be a mixture of two peptides which remained associated in 6 M guanidine and in 1% acetic acid, but were separated by SDS-polyacrylamide gel electrophoresis. The larger peptide had an Mr of 47 000 and gave low yields on end-group analysis; the smaller had an Mr of 4000 and was the C-terminal 36-residue fragment of alpha 1-antitrypsin. These results explain the consistent but erroneous finding of a reactive site near the N-terminus of alpha 1-antitrypsin, and confirm that the reactive site is 36 residues from the C-terminus.