By Magne K. Fagerhol, Diane Wilson Cox (auth.), Harry Harris, Kurt Hirschhorn (eds.)
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Similar renal changes have been reported by Milford Ward et al. (1975) and Sharp (1976). Glomerular renal damage, manifested by constant or recurrent proteinuria or hematuria, was found in 15% of the 246 Swedish Pi Z patients ascertained through hospital admission (Larsson, 1978). , 40 Magne K. Fagerhol and Diane Wilson Cox 1976a). these deposits were found in addition to those of immunoglobulins IgG, M, and A and complement (C3) which are frequently found in cases of membranoproliferative glomerulonephritis.
Laurell (1975) has suggested that uIAT, due to its small size and wide distribution in the extravascular fluid, can function as a shuttle for proteases. When such enzymes are released in the tissues, they are rapidly complexed with uIAT, and, later, the proteases are transferred to a second inhibitor, urmacroglobulin, which acts as a scavenger (Ohlsson, 1971a). The elastase-u2-macroglobulin complexes have a half-life in the Chapter 1: The Pi Polymorphism 27 circulation of about 12 min compared with 60 min for u\AT-elastase complexes in man (Ohlsson and Laurell, 1976).
Further direct studies of spermatozoa and the addition of pedigree data from other centers will be important for providing conclusions. BIOCHEMICAL ASPECTS A prerequisite for studies on the molecular structure of a protein is an efficient and nondegrading combination of methods for its purification. Unfortunately, u)AT has not been easy to purify. Early attempts included use of both low pH and high temperatures which, we now know, will denature the protein. The studies by Jirgensons (1977) have shown that the tertiary structure of u) AT is dependent upon proper hydration of parts 20 Magne K.