Alpha,-antitrypsin

CarrelJ, RW. and Travis, J. (1985). Alpha-1-antitrypsin and the serpins variation and countervariation. Trends Biochem. Sci. 10, 20-24. 

Alpha-1 Antitrypsin Deficiency Task Force. American Thoracic Society/European Respiratory Society Statement Standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med 2003 168 818-900. 

The basic genetic or biochemical defect that causes the condition (for example, alpha-1 antitrypsin deficiency)... 

Griffiths S.W., King J., and Cooney C.L. (2002), The reactivity and oxidation pathway of cysteine 232 in recombinant human alpha 1-antitrypsin, J. Biol. Chem. Ill, 25486-25492. 

Vemuri, S., C.D. Yu, and N. Roosdorp (1994). Effect of cryoprotectants on freezing, lyophilization, and storage of lyophilized recombinant alpha 1-antitrypsin formulations. PDA J Pharm Sci Technol 48(5) 241-246. 

The severe form of alpha-1 antitrypsin deficiency is the result of a single nucleotide substitution that produces a single amino acid substitution. This is best described as a... 

Alpha-1-antitrypsin Sheep fibrosis, other lung disorders... 

NTOl8 Churg, A., R. D. Wang, C. Xie, and J. L. Wright. Alpha-1-Antitrypsin ameliorates cigarette smoke-induced emphysema in the mouse. Am J Respir Crit Care Med 2003 168(2) 199-207. 

Rice suspension culture Alpha) 1 (-antitrypsin rAAT Trexler et al., 2005, Huang et al., 2001 3-12 mg/day, 200 mg/L Cyclical, semicontinuous culti system... 

Trexler, M.M., McDonald, K.A., and Jackman, A.P. (2005). A cyclical semicontin-uous process for production of human alpha 1 -antitrypsin using metabohcally induced plant cell suspension cultures. Biotechnol. Prog. 21(2) 321-328. 

Indications Replacement therapy in patients with congenital alpha-1 antitrypsin deficiency who have panacinar emphysema... 

Clinical pharmacology Alpha-1 antitrypsin deficiency is a chronic, hereditary, usually fatal, autosomal recessive disorder in which a low concentration of alphai-proteinase inhibitor is associated with slowly progressive, severe, panacinar emphysema that most often manifests itself in the third to fourth decades of fife. The pathogenesis of development of emphysema in alpha-1 antitrypsin deficiency is believed to be due to a chronic biochemical imbalance between elastase and alphai-proteinase inhibitor (the principal inhibitor of neutrophil elastase), which is deficient in alpha-1 antitrypsin disease. As a result it is believed that alveolar structures are unprotected from chronic exposure to elastase released from a chronic low-level burden of neutrophils in the lower respiratory tract, resulting in progressive degradation... 

G. Other consideration Alphai-proteinase inhibitor has been designated an orphan product for use as supplementary or replacement therapy for alpha-1 antitrypsin... 

Alpha-1 antitrypsin deficiency Cardiovascular diseases Familial hypercholesterolemia Many types using vaccine, toxin or endogenous regulatory protein expression strategies... 

Gilardi, P., Courtney, M., Pavirani, A., Perricaudet, M. (1990). Expression of human alpha 1-antitrypsin using a recombinant adenovirus vector. FEBS Lett., 267(1), 60-62. 

Rosenfeld, M.A., Siegfried, W., Yoshimura, K., et al. (1991). Adenovirus-mediated transfer of a recombinant alpha 1-antitrypsin gene to the lung epithelium in vivo. Science, 252(5004), 431 134. 

Mills K, Mills PB, Clayton PT, Mian N, Johnson AW, Winchester BG (2003) The underglyco-sylation of plasma alpha 1-antitrypsin in congenital disorders of glycosylation type I is not random. Glycobiology 13 73-85... 

Song S, Morgan M, Ellis T, Poirier A, et al. 1998. Sustained secretion of human-alpha-1 -antitrypsin from murine muscle transduced with adeno-associated virus vectors. Proc Nat Acad Sci. 95 14384-14388. 

Teckman, J.H., An, J. K., Blomenkamp, K., Schmidt, B., and Perlmutter, D., 2004, Mitochondrial autophagy and injury in the liver in alpha 1-antitrypsin deficiency, Am. J. Physiol. Gastrointest. Liver... 

Alpha 1-Antitrypsin Deficiency Biology Pathogenesis Clinical Manifestations Therapy, edited by R. G. Crystal... 

Alpha 1- antitrypsin (alA) Treatment of alA deficiency Sheep Aerosol Aerosolized alA able to pass through alveolar epithelium and gain access to interstitial compartment of lung [107]... 

G7. Gross, V., Scholmerich, J., Leser, H. G., Salm, R., Lausen, M., and Ruckauer, K., Granulocyte elastase in assessment of severity of acute pancreatitis. Comparison with acute-phase proteins C-reactive protein, alpha 1-antitrypsin, and protease inhibitor alpha 2-macroglobulin. Dig. Dis. Sci. 35, 97-105 (1990). 

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