The overall objective of the proposal is to investigate the structure, function and biosynthesis of glycoproteins involved directly in various human diseases. The specific areas of interest include: (1) serum a-l-antitrypsin variants (PiMM, PiZZ and PiSS) and pediatric cirrhosis, (2) lung glycoproteins in pulmonary alveolar proteinosis, (3) ceruloplasmin from normal serum and patients with Wilson's disease, and (4) isolation and characterization of sialyltransferases of human liver. Particular attention will be given to the oligosaccharide moieties of these glycoproteins. These studies should: (1) establish the structure of important complex glycoconjugates, (2) contribute to our understanding of the role of carbohydrate in glycoprotein synthesis and secretion and (3) lead to the establishment of improved therapeutic and diagnostic procedures. BIBLIOGRAPHIC REFERENCES: Kuhlenschmidt, M.S., Peters, S.P., Pinkard, O.D., Glew, R.H., and Sharp, H. "Cytidine-5'-Monophosphate-N-Acetylneuraminic Acid: Asialoglycoprotein Sialic Acid Transferase Activity in Liver and Serum of Patients with Juvenile Hepatic Cirrhosis and a-l-Antitrypsin Deficiency," Biochim. Biophys. Acta, 429-359-373 (1976). Yunis, E.J., Agostini, R.M., and Glew, R.H. "Fine Structural Observations of the Liver in Alpha-l-Antitrypsin Deficiency," Am. J. Pathol. 82:265-286 (1976).