Download Alpha-1 Antitrypsin: Role in Health and Disease by Adam Wanner, Robert A. Sandhaus PDF

By Adam Wanner, Robert A. Sandhaus

This publication bargains a accomplished review of alpha-1 antitrypsin deficiency, an inherited that results in lung ailment in adults and liver sickness in adults and youngsters and is linked to power obstructive lung illness in adults. whereas it's a infrequent , the mechanisms underlying the medical manifestations of this deficiency were principally clarified. remedy, notwithstanding, is obtainable just for the lung disorder that arises from the , hence necessitating endured study into new and replacement healing options. The ebook discusses the biology of alpha-1 antitrypsin, protein misfolding and polymerization, and analysis and remedy of alpha-1 antitrypsin deficiency and its linked ailments. It concludes with a dialogue of infrequent issues associated with alpha-1 antitrypsin deficiency and the position of healthcare organisations within the remedy of those illnesses. Written for pulmonary clinicians and scientists, Alpha-1 Antitrypsin: position in wellbeing and fitness and ailment is a necessary source that sheds gentle in this infrequent disease.

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Extra info for Alpha-1 Antitrypsin: Role in Health and Disease

Example text

Some examples include the use of AAT in the setting of inflammation and injury associated with diabetes mellitus, organ transplant rejection, reperfusion injury, asthma, and infectious diseases [77, 86, 91, 96, 100–106]. Summary In summary, a great deal has been learned about this protein since its discovery 50 years ago, but many important questions remain. There is increasing awareness of a functional role for AAT that extends beyond its capacity to inactivate serine proteases, but the import of this role in persons with either normal or abnormal AAT genotypes/phenotypes is not yet well understood.

1986;12:209–14. 36. Lai EC, Kao FT, Law ML, Woo SL. Assignment of the alpha 1-antitrypsin gene and a sequence-related gene to human chromosome 14 by molecular hybridization. Am J Hum Genet. 1983;35:385–92. 37. Long GL, Chandra T, Woo SL, Davie EW, Kurachi K. Complete sequence of the cDNA for human alpha 1-antitrypsin and the gene for the S variant. Biochemistry. 1984;23:4828–37. 38. De Simone V, Ciliberto G, Hardon E, Paonessa G, Palla F, Lundberg L, Cortese R. Cis- and trans-acting elements responsible for the cell-specific expression of the human alpha 1-antitrypsin gene.

Hadzic R, Nita IM, Tassidis H, Riesbeck K, Wingren AG, Janciauskiene S. Alpha1-antitrypsin inhibits Moraxella catarrhalis MID protein-induced tonsillar B cell proliferation and IL-6 release. Immunol Lett. 2006;102:141–7. 30 B. A. Wilson 102. Cantin AM, Woods DE. Aerosolized prolastin suppresses bacterial proliferation in a model of chronic Pseudomonas aeruginosa lung infection. Am J Respir Crit Care Med. 1999;160:1130–5. 103. Geraghty P, Rogan MP, Greene CM, Brantly ML, O'Neill SJ, Taggart CC, Mcelvaney NG.

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