4Dhami R,Gilks B,Xie C et al.Acute cigarette smoke-induced connective tissue breakdown is mediated by neurrophils and prevented by alphal-antitypsin.Am J Respir Cell Mol Biol,2000,22 (2):244
6Afdhal NH,Keaveny AP,Cohen SB et al.Urinary assays for desmosine and hydroxylysylpyridinoline in the detection of cirrhosis.J hepatol,1997,27(6):993
7Bode DC,Pagani ED,Cumiskey WR et al.Comparison of urinary desmosine excretion in patients with chronic obstructive pulmonary disease or cystic fibrosis.Pulm Pharmacol Ther,2000,13(4):175
8Serres FJ,Blanco I,Fernandez-Bustillo E.Genetic epidemiology of alpha-1 antitrypsin deficiency in North America and Australia/New Zealand:Australia,Canada,New Zealand and the United States of America.Clin Genet,2003,64(5):382
9Lang T,Muhlbauer M,Stuobelt M et al.Alpha-1-antitrypsin deficiency in children:liver disease is mot reflected by low serum levels of alpha-1-antitrypsin-a study on 48 pediatric patients.Eur J Med Res,2005,10(12):509
10Pittschieler K.Liver involvement in alphal-antitrypsin-deficient phenotypes PiSZ and PiMZ.Acta Paediatr,2002,91(2):239
3Crowther DC, Belorgey D, Miranda E, et al. Practical genetics: alpha-1-antitrypsin deficiency and the serpinopathies. Eur J Hum Genet, 2004,12: 167-172.
4Hersh CP, Dahl M, Ly NP, et al. Chronic obstructive pulmonary disease in alpha1-antitrypsin PI MZ heterozygotes: a meta-analysis. Thorax, 2004, 59: 843-849.
5Turino GM, Barker AF,Brandly ML,et al. Clinical features of individuals with PI*SZ phenotype of α1-antitrypsin deficiency. Am J Respir Crit Care Med, 1996,154:1718-1725.
6Miller SA, Dykes DD,Polesky HF. A simple salting out procedure for extracting DNA from human nucleated cells. Nucleic Acids Res, 1988,16:312-315.
7Braun A,Meyer P,CleveH,et al. Rapid and simple diagnosis of the common α1-proteinase inhibitor deficiency alleles Pi*Z and Pi*S by DNA analysis. Eur J Clin Chem Clin Biochem, 1996, 34: 761-764.
8Kirkham PA, Spooner G, Foulkes-Jones CF, et al. Cigarette smoke triggers macrophage adhesion and activation: role of lipid peroxidation products and scavenger receptor. Free Rad Bio Med, 2003,35:697-710.
3Stoller J K,Aboussouan L S.Alpha-1-antitrypsin deficien-cy[J].Lancet,2005,365(9478):2225-2236.
4Lang T,Muhlbauer M,Stuobelt M,et al.Alpha-1-antitryp-sin deficiency in children:liver disease is mot reflected by low serum levels of alpha-1-antitrypsin-a study on48pediat-ric patients[J].Eur J Med Res,2005,10(12):509-514.
5Dhami R,Gilks B,Xie C,et al.Acute cigarette smoke-induced connective tissue breakdown is mediated by neur-rophils and prevented byα1-antitypsin[J].Am J Respir Cell Mol Biol,2000,22(2):244-252.
6An J K,Blomenkamp K,Lindblad D,et al.Quantitative isolation of alpha-1-AT mutant Z protein polymers from hu-man and mouse livers and the effect of heat[J].Hepatolo-gy,2005,41(1):160-167.
9Patschull A O,Segu L,Nyon M P,et al.Therapeutic tar-get-site variability in alpha(1)-antitrypsin characterized at high resolution[J].Acta Crystallogr Sect F Struct Biol Cryst Commun,2011,67(12):1492-1497.
10Vukoti K M,Kadiyala C S,Miyagi M,et al.Streptomyces erythraeus trypsin inactivates alpha(1)-antitrypsin[J].FEBS Lett,2011,585(24):3898-3902.