PEDIATRICS Vol. 46 No. 6 December 1970, pp. 837-840
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ALPHA1-ANTITRYPSIN DEFICIENCY AND DISEASE

Chester A. Alper M.D.1 and A. Myron Johnson M.D.2

1 Blood Grouping Laboratory and Department of Medicine Children's Hospital Medical Center, Department of Pediatrics Harvard Medical School, 320 Longwood Avenue Boston, Massachusetts 02115
2 Blood Grouping Laboratory and Department of Medicine Children's Hospital Medical Center Department of Pediatrics Harvard Medical School, 320 Longwood Avenue Boston, Massachusetts 02115

The major serum inhibitor of proteolytic enzymes is the agr1-globulin, agr1-antitrypsin. Hereditary deficiency of this protein is immediately recognizable on inspection of stained serum electrophoretic strips (agarose, agar, and cellulose acetate, as well as paper) in that the normal agr1 band is absent (Fig. 1). Although the protein concentration of the agr1-globulin zone is usually subnormal in deficient sera, it is not always so; and, the ultimate criterion for the establishment of the diagnosis must be made by genetic typing, as will be shown.

Almost coincidental with the discovery of agr1-antitrypsin deficiency by Laurell and Eriksson,1 in Sweden, was their observation that many persons with this abnormality had early onset chronic obstructive lung disease.




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G. Francis, D. LaPointe, D. V. Eitzman, O. M. Rennert, and G. R. G. Monif
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