A-l-antitrypsine

Doring, G. 1999. Serine proteinase inhibitor therapy in a(l)-antitrypsin inhibitor deficiency and cystic fibrosis. Pediatr. Pul-monoX. 28 363-375. 

Histiocytic tumors Lysozyme, a-1-antitrypsin, a-l-antichymotrypsin, MAC-387, CD68... 

Poirier, A., Campbell-Thompson, M., Tang, Q., Scott-Jorgensen, M., Combee, L., Loiler, S., Crawford, J., Song, S. and Flotte, T. R. (2004). Toxicology and biodistribution studies of a recombinant adeno-associated virus 2-alpha-l antitrypsin vector. Preclinica 2, 43-51. 

Song, S., Morgan, M., Ellis, T., Poirier, A., Chesnut, K., Wang, J., Brantly, M., Muzyczka, N., Byrne, B. J., Atkinson, M. and Flotte, T. R. (1998). Sustained secretion of human alpha-l-antitrypsin from murine muscle transduced with adeno-associated virus vectors. Proc. Natl. Acad. Sci. USA 95, 14384-14388. 

Lomas, D.A., Mahadeva, R. Alpha(l)-antitrypsin polymerization and the serpinopathies pathobiology and prospects for therapy (review). J. Clin. Invest. 2002 110 1585-1590... 

Mowat, A.R Alpha-l-antitrypsin deficiency (PiZZ) features of liver involvement in childhood. Acta Paediatr. 1994 393 (Suppl.) 13-17... 

Prachalias, A.A., Kalife, M., Francavilla, R., Muiesan, R, Dhawan, A., Baker, A., Hadzic, D., Mieli-Vergani, G., Rela, M., Heaton, N.D. Liver transplantation for alpha-l-antitrypsin deficiency in children. Transplant. Internal. 2000 13 207—MO... 

Propst, T., Propst, A., Dietze, O., Judmaier, G., Braunsteiner, H., Vogel, W. Prevalence of hepatocellular carcinoma in alpha-l-antitrypsin deficiency. J. Hepatol. 1994 21 1006-1011... 

Fargion, S., Sergi, C., Bissoli, F., Fracanzani, A.L., Snlgo, E., Carraz-zone. A., Roberto, C., Cappellini, M.D., Fiorelli, G. Lack of association between porphyria cutanea tarda and -antitrypsin deficiency. Europ. J. Gastroenterol. Hepatol. 1996 8 387-391... 

Hubbard, R.C. Alpha-1 antitrypsin. In Inhalation Delivery of Therapeutic Peptides and Proteins Adjei, A.L., Gupta, P.R., Eds. Marcel Dekker, Inc. New York, 1997 315-330, Ch. 10. 

Byth BC, Billingsley GD, Cox DW. Physical and genetic mapping of the serpin gene cluster at 14q32.1 AUeUc association and a unique haplotype associated with alpha-l antitrypsin deficiency. Am J Hum Genet 1994 55 126-33. 

Scott CF, CarreH RW, Glaser CB, Kueppers F, Lewis JH, CoLman RW. Alpha-l-antitrypsin Pittsburgh a potent inhibitor of human plasma factor XIa, kaUrkrein, and factor Xllf. J CUn Invest I986 77 631-4. 

Materials such as soybeans, lima beans, and egg whites contain natural TRY inhibitors—small polypeptides such as tti-antitrypsin (a-l-protease inhibitor) and a2-macroglob-uUn— that combine irreversibly with TRY and inactivate it by blocldng the active center. Similar nondialyzable TRY inhibitors are present in pancreatic juice, serum, and urine. These inhibitors protect plasma and other proteins against hydrolysis by TRY and other proteases if for some reason any appreciable quantity of the enzyme enters the vascular system. The absence of ai-antitrypsin is associated with an increased tendency toward panlobular emphysema in early hfe this example illustrates the effects of uninhibited proteases on organ function. 

Propst T, Propst A, Dietze O, Judmaier G, Braun-steiner H, Vogel W. Alpha-l-antitrypsin deficiency and liver disease. Dig Dis 1994 12 139-49. 

Terashima, M. Murai, Y. Kawamura, M. Nakanishi, S. Stoltz, T. Chen, L. Drohan, W. Rodriguez, R.L. Katoh, S. Production of functional human a 1-antitrypsin by plant cell culture. Appl. Microbiol. Biotechnol. 1999, 52, 516-523. 

The granular cell tumor (GCT) is an uncommon tumor of Schwann cell origin with a predilection for skin and mucosal surfaces, especially the tongue. Most are benign, but a few are malignant. The tumor is positive for S-100 protein, neuron specific enolase, alpha-l-antitrypsin, CD 68, vimentin, inhibin-alpha, protein gene product... 

Beckman G, Beckman L, Bjelle A, et al. Alpha-l-antitrypsin types and rheumatoid arthritis. Clin Genet 1984 25(6) 496-499. 

Observational studies In a multicenter, observational retrospective review of 127 patients (80 male, mean age 51.7years) who had undergone at least ISmonths of treatment with alpha-l-antitrypsin therapy (Trypsone or Pro-lastin ), there were four serious adverse events that occurred during the treatment period one massive pulmonary thromboembolism, one myeloid leukaemia, one acute myocardial infarction and one haemorrhagic infarcHon but were judged not to be related to treatment. Seven of 11 nonserious adverse events were considered related to treatment including mild to moderate cutaneous symptoms (facial erythema, oedema/pruritus, rash), chills, fever and anxiety [31 ]. 

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