-
1
-
-
0028206262
-
The serpin superfamily of proteinase inhibitors: Structure, function, and regulation
-
23
-
Potempa J, Korzus E, Travis J (1994) The serpin superfamily of proteinase inhibitors: structure, function, and regulation. J Biol Chem 269(23):15957-15960
-
(1994)
J Biol Chem
, vol.269
, pp. 15957-15960
-
-
Potempa, J.1
Korzus, E.2
Travis, J.3
-
2
-
-
0001724344
-
Serpins: The superfamily of serine proteinase inhibitors
-
Elsevier Biomedical Press Amsterdam
-
Carrell RW (1986) Serpins: the superfamily of serine proteinase inhibitors. In: Barrett A (eds) Proteinase Inhibitors. Amsterdam: Elsevier Biomedical Press, pp 403-419
-
(1986)
Proteinase Inhibitors
, pp. 403-419
-
-
Carrell, R.W.1
Barrett, A.2
-
3
-
-
0021076101
-
The isolation of a clone for human alpha 1-antitrypsin and the detection of alpha 1-antitrypsin in mRNA from liver and leukocytes
-
2
-
Rogers J, Kalsheker N, Wallis S, Speer A, Coutelle CH, Woods D, Humphries SE (1983) The isolation of a clone for human alpha 1-antitrypsin and the detection of alpha 1-antitrypsin in mRNA from liver and leukocytes. Biochem Biophys Res Commun 116(2):375-382
-
(1983)
Biochem Biophys Res Commun
, vol.116
, pp. 375-382
-
-
Rogers, J.1
Kalsheker, N.2
Wallis, S.3
Speer, A.4
Coutelle, C.H.5
Woods, D.6
Humphries, S.E.7
-
4
-
-
0023689993
-
Multiple tissues express alpha 1-antitrypsin in transgenic mice and man
-
1
-
Carlson JA, Rogers BB, Sifers RN, Hawkins HK, Finegold MJ, Woo SL (1988) Multiple tissues express alpha 1-antitrypsin in transgenic mice and man. J Clin Invest 82(1):26-36
-
(1988)
J Clin Invest
, vol.82
, pp. 26-36
-
-
Carlson, J.A.1
Rogers, B.B.2
Sifers, R.N.3
Hawkins, H.K.4
Finegold, M.J.5
Woo, S.L.6
-
5
-
-
0027361105
-
Cell-specific expression of alpha 1-antitrypsin in human intestinal epithelium
-
4
-
Molmenti EP, Perlmutter DH, Rubin DC (1993) Cell-specific expression of alpha 1-antitrypsin in human intestinal epithelium. J Clin Invest 92(4):2022-2034
-
(1993)
J Clin Invest
, vol.92
, pp. 2022-2034
-
-
Molmenti, E.P.1
Perlmutter, D.H.2
Rubin, D.C.3
-
6
-
-
0021147683
-
Leukocyte kinetics in the human lung: Role of exercise and catecholamines
-
3
-
Muir AL, Cruz M, Martin BA, Thommasen H, Belzberg A, Hogg JC (1984) Leukocyte kinetics in the human lung: role of exercise and catecholamines. J Appl Physiol 57(3):711-719
-
(1984)
J Appl Physiol
, vol.57
, pp. 711-719
-
-
Muir, A.L.1
Cruz, M.2
Martin, B.A.3
Thommasen, H.4
Belzberg, A.5
Hogg, J.C.6
-
7
-
-
0021941090
-
Elastase in tissue injury
-
Janoff A (1985) Elastase in tissue injury. Annu Rev Med 36:207-216
-
(1985)
Annu Rev Med
, vol.36
, pp. 207-216
-
-
Janoff, A.1
-
8
-
-
2642584282
-
A canine model for production of severe unilateral panacinar emphysema
-
4
-
Chino K, Choong CK, Toeniskoetter PD, Cooper JD, Lausberg HF, Bae KT, Pierce JA, Hogg JC (2004) A canine model for production of severe unilateral panacinar emphysema. Exp Lung Res 30(4):319-332
-
(2004)
Exp Lung Res
, vol.30
, pp. 319-332
-
-
Chino, K.1
Choong, C.K.2
Toeniskoetter, P.D.3
Cooper, J.D.4
Lausberg, H.F.5
Bae, K.T.6
Pierce, J.A.7
Hogg, J.C.8
-
9
-
-
0019369133
-
Emphysema induced in vitro and in vivo in dogs by a purified elastase from homologous leukocytes
-
3
-
Sloan B, Abrams WR, Meranze DR, Kimbel P, Weinbaum G (1981) Emphysema induced in vitro and in vivo in dogs by a purified elastase from homologous leukocytes. Am Rev Respir Dis 124(3):295-301
-
(1981)
Am Rev Respir Dis
, vol.124
, pp. 295-301
-
-
Sloan, B.1
Abrams, W.R.2
Meranze, D.R.3
Kimbel, P.4
Weinbaum, G.5
-
10
-
-
0031893793
-
Alpha 1-antitrypsin and protease complexation is induced by lipopolysaccharide, interleukin-1beta, and tumor necrosis factor-alpha in monocytes
-
1
-
Knoell DL, Ralston DR, Coulter KR, Wewers MD (1998) Alpha 1-antitrypsin and protease complexation is induced by lipopolysaccharide, interleukin-1beta, and tumor necrosis factor-alpha in monocytes. Am J Respir Crit Care Med 157(1):246-255
-
(1998)
Am J Respir Crit Care Med
, vol.157
, pp. 246-255
-
-
Knoell, D.L.1
Ralston, D.R.2
Coulter, K.R.3
Wewers, M.D.4
-
11
-
-
0025393913
-
Epidemiology of alpha 1-protease inhibitor deficiency
-
Hutchison DC (1990) Epidemiology of alpha 1-protease inhibitor deficiency. Eur Respir J Suppl 9:29s-34s
-
(1990)
Eur Respir J Suppl
, vol.9
-
-
Hutchison, D.C.1
-
12
-
-
0036433482
-
Worldwide racial and ethnic distribution of alpha1-antitrypsin deficiency: Summary of an analysis of published genetic epidemiologic surveys
-
5
-
de Serres FJ (2002) Worldwide racial and ethnic distribution of alpha1-antitrypsin deficiency: summary of an analysis of published genetic epidemiologic surveys. Chest 122(5):1818-1829
-
(2002)
Chest
, vol.122
, pp. 1818-1829
-
-
De Serres, F.J.1
-
13
-
-
0024397003
-
Serum alpha 1-antitrypsin deficiency associated with the common S-type (Glu264-Val) mutation results from intracellular degradation of alpha 1-antitrypsin prior to secretion
-
18
-
Curiel DT, Chytil A, Courtney M, Crystal RG (1989) Serum alpha 1-antitrypsin deficiency associated with the common S-type (Glu264-Val) mutation results from intracellular degradation of alpha 1-antitrypsin prior to secretion. J Biol Chem 264(18):10477-10486
-
(1989)
J Biol Chem
, vol.264
, pp. 10477-10486
-
-
Curiel, D.T.1
Chytil, A.2
Courtney, M.3
Crystal, R.G.4
-
14
-
-
0029907210
-
Structural explanation for the deficiency of S alpha 1-antitrypsin
-
11
-
Elliott PR, Stein PE, Bilton D, Carrell RW, Lomas DA (1996) Structural explanation for the deficiency of S alpha 1-antitrypsin. Nat Struct Biol 3(11):910-911
-
(1996)
Nat Struct Biol
, vol.3
, pp. 910-911
-
-
Elliott, P.R.1
Stein, P.E.2
Bilton, D.3
Carrell, R.W.4
Lomas, D.A.5
-
15
-
-
0026755363
-
The mechanism of Z alpha 1-antitrypsin accumulation in the liver
-
6379
-
Lomas DA, Evans DL, Finch JT, Carrell RW (1992) The mechanism of Z alpha 1-antitrypsin accumulation in the liver. Nature 357(6379):605-607
-
(1992)
Nature
, vol.357
, pp. 605-607
-
-
Lomas, D.A.1
Evans, D.L.2
Finch, J.T.3
Carrell, R.W.4
-
16
-
-
0032065494
-
Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema
-
5
-
Elliott PR, Bilton D, Lomas DA (1998) Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema. Am J Respir Cell Mol Biol 18(5):670-674
-
(1998)
Am J Respir Cell Mol Biol
, vol.18
, pp. 670-674
-
-
Elliott, P.R.1
Bilton, D.2
Lomas, D.A.3
-
17
-
-
0037135563
-
Detection of circulating and endothelial cell polymers of Z and wild type alpha 1-antitrypsin by a monoclonal antibody
-
29
-
Janciauskiene S, Dominaitiene R, Stemby NH, Piitulainen E, Eriksson S (2002) Detection of circulating and endothelial cell polymers of Z and wild type alpha 1-antitrypsin by a monoclonal antibody. J Biol Chem 277(29):26540-26546
-
(2002)
J Biol Chem
, vol.277
, pp. 26540-26546
-
-
Janciauskiene, S.1
Dominaitiene, R.2
Stemby, N.H.3
Piitulainen, E.4
Eriksson, S.5
-
18
-
-
0025351137
-
Decline in pulmonary function in patients with alpha 1-antitrypsin deficiency
-
Evald T, Dirksen A, Keittelmann S, Viskum K, Kok-Jensen A (1990) Decline in pulmonary function in patients with alpha 1-antitrypsin deficiency. Lung 168 Suppl:579-585
-
(1990)
Lung 168
, pp. 579-585
-
-
Evald, T.1
Dirksen, A.2
Keittelmann, S.3
Viskum, K.4
Kok-Jensen, A.5
-
19
-
-
0018128288
-
Natural history and life expectancy in severe alpha1-antitrypsin deficiency, Pi Z
-
5
-
Larsson C (1978) Natural history and life expectancy in severe alpha1-antitrypsin deficiency, Pi Z. Acta Med Scand 204(5):345-351
-
(1978)
Acta Med Scand
, vol.204
, pp. 345-351
-
-
Larsson, C.1
-
20
-
-
0016097113
-
Alpha1-antitrypsin and its deficiency
-
2
-
Kueppers F, Black LF (1974) Alpha1-antitrypsin and its deficiency. Am Rev Respir Dis 110(2):176-194
-
(1974)
Am Rev Respir Dis
, vol.110
, pp. 176-194
-
-
Kueppers, F.1
Black, L.F.2
-
21
-
-
0023795389
-
Clinical features and history of the destructive lung disease associated with alpha-1-antitrypsin deficiency of adults with pulmonary symptoms
-
2
-
Brantly ML, Paul LD, Miller BH, Falk RT, Wu M, Crystal RG (1988) Clinical features and history of the destructive lung disease associated with alpha-1-antitrypsin deficiency of adults with pulmonary symptoms. Am Rev Respir Dis 138(2):327-336
-
(1988)
Am Rev Respir Dis
, vol.138
, pp. 327-336
-
-
Brantly, M.L.1
Paul, L.D.2
Miller, B.H.3
Falk, R.T.4
Wu, M.5
Crystal, R.G.6
-
22
-
-
0027400969
-
Variants of alpha 1-antitrypsin in Puerto Rican children with asthma
-
3
-
Colp C, Pappas J, Moran D, Lieberman J (1993) Variants of alpha 1-antitrypsin in Puerto Rican children with asthma. Chest 103(3):812-815
-
(1993)
Chest
, vol.103
, pp. 812-815
-
-
Colp, C.1
Pappas, J.2
Moran, D.3
Lieberman, J.4
-
23
-
-
0028454320
-
Absence of alpha-1-antitrypsin (Pi Null Bellingham) and the early onset of emphysema
-
3
-
Cook L, Janus ED, Brenton S, Tai E, Burdon J (1994) Absence of alpha-1-antitrypsin (Pi Null Bellingham) and the early onset of emphysema. Aust N Z J Med 24(3):263-269
-
(1994)
Aust N Z J Med
, vol.24
, pp. 263-269
-
-
Cook, L.1
Janus, E.D.2
Brenton, S.3
Tai, E.4
Burdon, J.5
-
24
-
-
0024434432
-
Alpha-1-antitrypsin deficiency. High prevalence in the St. Louis area determined by direct population screening
-
4
-
Silverman EK, Miletich JP, Pierce JA, Sherman LA, Endicott SK, Broze GJ Jr, Campbell EJ (1989) Alpha-1-antitrypsin deficiency. High prevalence in the St. Louis area determined by direct population screening. Am Rev Respir Dis 140(4):961-966
-
(1989)
Am Rev Respir Dis
, vol.140
, pp. 961-966
-
-
Silverman, E.K.1
Miletich, J.P.2
Pierce, J.A.3
Sherman, L.A.4
Endicott, S.K.5
Broze Jr., G.J.6
Campbell, E.J.7
-
25
-
-
0017064849
-
[Clinical, clinical-chemical and pathological findings in familial alpha1-antitrypsin deficiency]
-
5
-
Christen H, Henke J (1976) [Clinical, clinical-chemical and pathological findings in familial alpha1-antitrypsin deficiency]. Monatsschr Kinderheilkd 124(5):384-386
-
(1976)
Monatsschr Kinderheilkd
, vol.124
, pp. 384-386
-
-
Christen, H.1
Henke, J.2
-
26
-
-
0019963490
-
Alpha 1-antitrypsin deficiency: The radiological features of pulmonary emphysema in subjects of Pi type Z and Pi type SZ: A survey by the British Thoracic Association
-
4
-
Gishen P, Saunders AJ, Tobin MJ, Hutchison DC (1982) Alpha 1-antitrypsin deficiency: the radiological features of pulmonary emphysema in subjects of Pi type Z and Pi type SZ: a survey by the British Thoracic Association. Clin Radiol 33(4):371-377
-
(1982)
Clin Radiol
, vol.33
, pp. 371-377
-
-
Gishen, P.1
Saunders, A.J.2
Tobin, M.J.3
Hutchison, D.C.4
-
27
-
-
0020678269
-
Alpha 1 antitrypsin deficiency: The clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type Z. a survey by the British Thoracic Association
-
1
-
Tobin MJ, Cook PJ, Hutchison DC (1983) Alpha 1 antitrypsin deficiency: the clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type Z. A survey by the British Thoracic Association. Br J Dis Chest 77(1):14-27
-
(1983)
Br J Dis Chest
, vol.77
, pp. 14-27
-
-
Tobin, M.J.1
Cook, P.J.2
Hutchison, D.C.3
-
28
-
-
0025056573
-
Long-term follow-up of a cohort of children with alpha-1-antitrypsin deficiency
-
2
-
Wall M, Moe E, Eisenberg J, Powers M, Buist N, Buist AS (1990) Long-term follow-up of a cohort of children with alpha-1-antitrypsin deficiency. J Pediatr 116(2):248-251
-
(1990)
J Pediatr
, vol.116
, pp. 248-251
-
-
Wall, M.1
Moe, E.2
Eisenberg, J.3
Powers, M.4
Buist, N.5
Buist, A.S.6
-
29
-
-
0020669992
-
The natural history of air-flow obstruction in PiZ emphysema. Report of an NHLBI workshop
-
2
-
Buist AS, Burrows B, Eriksson S, Mittman C, Wu M (1983) The natural history of air-flow obstruction in PiZ emphysema. Report of an NHLBI workshop. Am Rev Respir Dis 127(2):S43-S45
-
(1983)
Am Rev Respir Dis
, vol.127
-
-
Buist, A.S.1
Burrows, B.2
Eriksson, S.3
Mittman, C.4
Wu, M.5
-
30
-
-
0021960082
-
Smoking, lung function, and alpha 1-antitrypsin deficiency
-
8421
-
Janus ED, Phillips NT, Carrell RW (1985) Smoking, lung function, and alpha 1-antitrypsin deficiency. Lancet 1(8421):152-154
-
(1985)
Lancet
, vol.1
, pp. 152-154
-
-
Janus, E.D.1
Phillips, N.T.2
Carrell, R.W.3
-
31
-
-
0024211686
-
Lung function, smoking and survival in severe alpha 1-antitrypsin deficiency, PiZZ
-
12
-
Wu MC, Eriksson S (1988) Lung function, smoking and survival in severe alpha 1-antitrypsin deficiency, PiZZ. J Clin Epidemiol 41(12):1157-1165
-
(1988)
J Clin Epidemiol
, vol.41
, pp. 1157-1165
-
-
Wu, M.C.1
Eriksson, S.2
-
33
-
-
0026551587
-
High resolution computed tomography (HRCT) in emphysema associated with alpha-1-antitrypsin deficiency
-
4
-
Guest PJ, Hansell DM (1992) High resolution computed tomography (HRCT) in emphysema associated with alpha-1-antitrypsin deficiency. Clin Radiol 45(4):260-266
-
(1992)
Clin Radiol
, vol.45
, pp. 260-266
-
-
Guest, P.J.1
Hansell, D.M.2
-
34
-
-
0027519368
-
Bronchiectasis in patients with alpha 1-antitrypsin deficiency
-
5
-
Shin MS, Ho KJ (1993) Bronchiectasis in patients with alpha 1-antitrypsin deficiency. A rare occurrence? Chest 104(5):1384-1386
-
(1993)
A Rare Occurrence? Chest
, vol.104
, pp. 1384-1386
-
-
Shin, M.S.1
Ho, K.J.2
-
35
-
-
0029963017
-
Alpha 1-antitrypsin deficiency: Evaluation of bronchiectasis with CT
-
1
-
King MA, Stone JA, Diaz PT, Mueller CF, Becker WJ, Gadek JE (1996) Alpha 1-antitrypsin deficiency: evaluation of bronchiectasis with CT. Radiology 199(1):137-141
-
(1996)
Radiology
, vol.199
, pp. 137-141
-
-
King, M.A.1
Stone, J.A.2
Diaz, P.T.3
Mueller, C.F.4
Becker, W.J.5
Gadek, J.E.6
-
36
-
-
0031225933
-
Progress of emphysema in severe alpha 1-antitrypsin deficiency as assessed by annual CT
-
5
-
Dirksen A, Friis M, Olesen KP, Skovgaard LT, Sorensen K (1997) Progress of emphysema in severe alpha 1-antitrypsin deficiency as assessed by annual CT. Acta Radiol 38(5):826-832
-
(1997)
Acta Radiol
, vol.38
, pp. 826-832
-
-
Dirksen, A.1
Friis, M.2
Olesen, K.P.3
Skovgaard, L.T.4
Sorensen, K.5
-
37
-
-
4444382577
-
Short-term reproducibility of computed tomography-based lung density measurements in alpha-1 antitrypsin deficiency and smokers with emphysema
-
4
-
Shaker SB, Dirksen A, Laursen LC, Maltbaek N, Christensen L, Sander U, Seersholm N, Skovgaard LT, Nielsen L, Kok-Jensen A (2004) Short-term reproducibility of computed tomography-based lung density measurements in alpha-1 antitrypsin deficiency and smokers with emphysema. Acta Radiol 45(4):424-430
-
(2004)
Acta Radiol
, vol.45
, pp. 424-430
-
-
Shaker, S.B.1
Dirksen, A.2
Laursen, L.C.3
Maltbaek, N.4
Christensen, L.5
Sander, U.6
Seersholm, N.7
Skovgaard, L.T.8
Nielsen, L.9
Kok-Jensen, A.10
-
38
-
-
0022637178
-
Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency
-
12
-
Eriksson S, Carlson J, Velez R (1986) Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency. N Engl J Med 314(12):736-739
-
(1986)
N Engl J Med
, vol.314
, pp. 736-739
-
-
Eriksson, S.1
Carlson, J.2
Velez, R.3
-
39
-
-
0024238708
-
The natural history of liver disease in alpha 1-antitrypsin deficient children
-
6
-
Sveger T (1988) The natural history of liver disease in alpha 1-antitrypsin deficient children. Acta Paediatr Scand 77(6):847-851
-
(1988)
Acta Paediatr Scand
, vol.77
, pp. 847-851
-
-
Sveger, T.1
-
41
-
-
0021029558
-
Outcome of liver disease associated with alpha 1 antitrypsin deficiency (PiZ). Implications for genetic counselling and antenatal diagnosis
-
11
-
Psacharopoulos HT, Mowat AP, Cook PJ, Carlile PA, Portmann B, Rodeck CH (1983) Outcome of liver disease associated with alpha 1 antitrypsin deficiency (PiZ). Implications for genetic counselling and antenatal diagnosis. Arch Dis Child 58(11):882-887
-
(1983)
Arch Dis Child
, vol.58
, pp. 882-887
-
-
Psacharopoulos, H.T.1
Mowat, A.P.2
Cook, P.J.3
Carlile, P.A.4
Portmann, B.5
Rodeck, C.H.6
-
42
-
-
0020698177
-
Risk for liver disease in adults with alpha 1-antitrypsin deficiency
-
2
-
Cox DW, Smyth S (1983) Risk for liver disease in adults with alpha 1-antitrypsin deficiency. Am J Med 74(2):221-227
-
(1983)
Am J Med
, vol.74
, pp. 221-227
-
-
Cox, D.W.1
Smyth, S.2
-
43
-
-
0016331794
-
Hepatocyte ultrastructural changes in alpha1-antitrypsin deficiency
-
6
-
Feldmann G, Bignon J, Chahinian P, Degott C, Benhamou JP (1974) Hepatocyte ultrastructural changes in alpha1-antitrypsin deficiency. Gastroenterology 67(6):1214-1224
-
(1974)
Gastroenterology
, vol.67
, pp. 1214-1224
-
-
Feldmann, G.1
Bignon, J.2
Chahinian, P.3
Degott, C.4
Benhamou, J.P.5
-
44
-
-
0019780071
-
Alpha-1-Antitrypsin phenotypes in hepatocellular carcinoma
-
6
-
Govindarajan S, Ashcavai M, Peters RL (1981) Alpha-1-Antitrypsin phenotypes in hepatocellular carcinoma. Hepatology 1(6):628-631
-
(1981)
Hepatology
, vol.1
, pp. 628-631
-
-
Govindarajan, S.1
Ashcavai, M.2
Peters, R.L.3
-
45
-
-
33748062353
-
Pathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency
-
2
-
Perlmutter DH (2006) Pathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency. Pediatr Res 60(2):233-238
-
(2006)
Pediatr Res
, vol.60
, pp. 233-238
-
-
Perlmutter, D.H.1
-
46
-
-
0017099344
-
Liver disease in alpha1-antitrypsin deficiency detected by screening of 200,000 infants
-
24
-
Sveger T (1976) Liver disease in alpha1-antitrypsin deficiency detected by screening of 200,000 infants. N Engl J Med 294(24):1316-1321
-
(1976)
N Engl J Med
, vol.294
, pp. 1316-1321
-
-
Sveger, T.1
-
47
-
-
0029083915
-
The liver in adolescents with alpha 1-antitrypsin deficiency
-
2
-
Sveger T, Eriksson S (1995) The liver in adolescents with alpha 1-antitrypsin deficiency. Hepatology 22(2):514-517
-
(1995)
Hepatology
, vol.22
, pp. 514-517
-
-
Sveger, T.1
Eriksson, S.2
-
48
-
-
0028593988
-
A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency
-
19
-
Wu Y, Whitman I, Molmenti E, Moore K, Hippenmeyer P, Perlmutter DH (1994) A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency. Proc Natl Acad Sci U S A 91(19):9014-9018
-
(1994)
Proc Natl Acad Sci U S a
, vol.91
, pp. 9014-9018
-
-
Wu, Y.1
Whitman, I.2
Molmenti, E.3
Moore, K.4
Hippenmeyer, P.5
Perlmutter, D.H.6
-
49
-
-
0025242929
-
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
-
4
-
Cheng SH, Gregory RJ, Marshall J, Paul S, Souza DW, White GA, O'Riordan CR, Smith AE (1990) Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 63(4):827-834
-
(1990)
Cell
, vol.63
, pp. 827-834
-
-
Cheng, S.H.1
Gregory, R.J.2
Marshall, J.3
Paul, S.4
Souza, D.W.5
White, G.A.6
O'Riordan, C.R.7
Smith, A.E.8
-
50
-
-
0026795608
-
3-Hydroxy-3-methylglutaryl-coenzyme a reductase and T cell receptor alpha subunit are differentially degraded in the endoplasmic reticulum
-
13
-
Inoue S, Simoni RD (1992) 3-Hydroxy-3-methylglutaryl-coenzyme A reductase and T cell receptor alpha subunit are differentially degraded in the endoplasmic reticulum. J Biol Chem 267(13):9080-9086
-
(1992)
J Biol Chem
, vol.267
, pp. 9080-9086
-
-
Inoue, S.1
Simoni, R.D.2
-
51
-
-
0029788023
-
Degradation of a mutant secretory protein, alpha1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity
-
37
-
Qu D, Teckman JH, Omura S, Perlmutter DH (1996) Degradation of a mutant secretory protein, alpha1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity. J Biol Chem 271(37):22791-22795
-
(1996)
J Biol Chem
, vol.271
, pp. 22791-22795
-
-
Qu, D.1
Teckman, J.H.2
Omura, S.3
Perlmutter, D.H.4
-
52
-
-
0035976919
-
The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes
-
48
-
Teckman JH, Burrows J, Hidvegi T, Schmidt B, Hale PD, Perlmutter DH (2001) The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes. J Biol Chem 276(48):44865-44872
-
(2001)
J Biol Chem
, vol.276
, pp. 44865-44872
-
-
Teckman, J.H.1
Burrows, J.2
Hidvegi, T.3
Schmidt, B.4
Hale, P.D.5
Perlmutter, D.H.6
-
53
-
-
0032526051
-
Inhibition of intracellular degradation increases secretion of a mutant form of alpha1-antitrypsin associated with profound deficiency
-
12
-
Novoradovskaya N, Lee J, Yu ZX, Ferrans VJ, Brantly M (1998) Inhibition of intracellular degradation increases secretion of a mutant form of alpha1-antitrypsin associated with profound deficiency. J Clin Invest 101(12):2693-2701
-
(1998)
J Clin Invest
, vol.101
, pp. 2693-2701
-
-
Novoradovskaya, N.1
Lee, J.2
Yu, Z.X.3
Ferrans, V.J.4
Brantly, M.5
-
54
-
-
0034637555
-
Processing by endoplasmic reticulum mannosidases partitions a secretion-impaired glycoprotein into distinct disposal pathways
-
32
-
Cabral CM, Choudhury P, Liu Y, Sifers RN (2000) Processing by endoplasmic reticulum mannosidases partitions a secretion-impaired glycoprotein into distinct disposal pathways. J Biol Chem 275(32):25015-25022
-
(2000)
J Biol Chem
, vol.275
, pp. 25015-25022
-
-
Cabral, C.M.1
Choudhury, P.2
Liu, Y.3
Sifers, R.N.4
-
55
-
-
0034724520
-
Functional and genomic analyses reveal an essential coordination between the unfolded protein response and ER-associated degradation
-
3
-
Travers KJ, Patil CK, Wodicka L, Lockhart DJ, Weissman JS, Walter P (2000) Functional and genomic analyses reveal an essential coordination between the unfolded protein response and ER-associated degradation. Cell 101(3):249-258
-
(2000)
Cell
, vol.101
, pp. 249-258
-
-
Travers, K.J.1
Patil, C.K.2
Wodicka, L.3
Lockhart, D.J.4
Weissman, J.S.5
Walter, P.6
-
56
-
-
0033780610
-
A regulatory link between ER-associated protein degradation and the unfolded-protein response
-
7
-
Friedlander R, Jarosch E, Urban J, Volkwein C, Sommer T (2000) A regulatory link between ER-associated protein degradation and the unfolded-protein response. Nat Cell Biol 2(7):379-384
-
(2000)
Nat Cell Biol
, vol.2
, pp. 379-384
-
-
Friedlander, R.1
Jarosch, E.2
Urban, J.3
Volkwein, C.4
Sommer, T.5
-
57
-
-
0023942723
-
Alpha-1-antitrypsin deficiency and panniculitis. Perspectives on disease relationship and replacement therapy
-
6A
-
Pittelkow MR, Smith KC, Su WP (1988) Alpha-1-antitrypsin deficiency and panniculitis. Perspectives on disease relationship and replacement therapy. Am J Med 84(6A):80-86
-
(1988)
Am J Med
, vol.84
, pp. 80-86
-
-
Pittelkow, M.R.1
Smith, K.C.2
Su, W.P.3
-
58
-
-
0020662195
-
Membranoproliferative glomerulonephritis and alpha 1-antitrypsin deficiency in children
-
1
-
Strife CF, Hug G, Chuck G, McAdams AJ, Davis CA, Kline JJ (1983) Membranoproliferative glomerulonephritis and alpha 1-antitrypsin deficiency in children. Pediatrics 71(1):88-92
-
(1983)
Pediatrics
, vol.71
, pp. 88-92
-
-
Strife, C.F.1
Hug, G.2
Chuck, G.3
McAdams, A.J.4
Davis, C.A.5
Kline, J.J.6
-
59
-
-
0025690316
-
Liver disease in alpha-1-antitrypsin deficiency: Prognostic indicators
-
6
-
Ibarguen E, Gross CR, Savik SK, Sharp HL (1990) Liver disease in alpha-1-antitrypsin deficiency: prognostic indicators. J Pediatr 117(6):864-870
-
(1990)
J Pediatr
, vol.117
, pp. 864-870
-
-
Ibarguen, E.1
Gross, C.R.2
Savik, S.K.3
Sharp, H.L.4
-
60
-
-
0027988324
-
Alpha 1-antitrypsin: A guardian of vascular tissue
-
11
-
Cox DW (1994) Alpha 1-antitrypsin: a guardian of vascular tissue. Mayo Clin Proc 69(11):1123-1124
-
(1994)
Mayo Clin Proc
, vol.69
, pp. 1123-1124
-
-
Cox, D.W.1
-
61
-
-
0028173310
-
Strong link between the alpha 1-antitrypsin PiZ allele and Wegener's granulomatosis
-
5
-
Elzouki AN, Segelmark M, Wieslander J, Eriksson S (1994) Strong link between the alpha 1-antitrypsin PiZ allele and Wegener's granulomatosis. J Intern Med 236(5):543-548
-
(1994)
J Intern Med
, vol.236
, pp. 543-548
-
-
Elzouki, A.N.1
Segelmark, M.2
Wieslander, J.3
Eriksson, S.4
-
62
-
-
0034979113
-
Anti-proteinase 3 antibody activation of neutrophils can be inhibited by alpha1-antitrypsin
-
6
-
Rooney CP, Taggart C, Coakley R, McElvaney NG, O'Neill SJ (2001) Anti-proteinase 3 antibody activation of neutrophils can be inhibited by alpha1-antitrypsin. Am J Respir Cell Mol Biol 24(6):747-754
-
(2001)
Am J Respir Cell Mol Biol
, vol.24
, pp. 747-754
-
-
Rooney, C.P.1
Taggart, C.2
Coakley, R.3
McElvaney, N.G.4
O'Neill, S.J.5
-
63
-
-
0016716501
-
Liver, lung and malignant disease in heterozygous (Pi MZ) alpha1-antitrypsin deficiency
-
4
-
Eriksson S, Moestrup T, Hagerstrand I (1975) Liver, lung and malignant disease in heterozygous (Pi MZ) alpha1-antitrypsin deficiency. Acta Med Scand 198(4):243-247
-
(1975)
Acta Med Scand
, vol.198
, pp. 243-247
-
-
Eriksson, S.1
Moestrup, T.2
Hagerstrand, I.3
-
64
-
-
0037011795
-
Alpha1-antitrypsin deficiency-a model for conformational diseases
-
1
-
Carrell RW, Lomas DA (2002) Alpha1-antitrypsin deficiency-a model for conformational diseases. N Engl J Med 346(1):45-53
-
(2002)
N Engl J Med
, vol.346
, pp. 45-53
-
-
Carrell, R.W.1
Lomas, D.A.2
-
65
-
-
0036301160
-
Familial conformational diseases and dementias
-
1
-
Crowther DC (2002) Familial conformational diseases and dementias. Hum Mutat 20(1):1-14
-
(2002)
Hum Mutat
, vol.20
, pp. 1-14
-
-
Crowther, D.C.1
-
66
-
-
0030610007
-
Biosynthesis of alpha1-proteinase inhibitor by human lung-derived epithelial cells
-
13
-
Cichy J, Potempa J, Travis J (1997) Biosynthesis of alpha1-proteinase inhibitor by human lung-derived epithelial cells. J Biol Chem 272(13):8250-8255
-
(1997)
J Biol Chem
, vol.272
, pp. 8250-8255
-
-
Cichy, J.1
Potempa, J.2
Travis, J.3
-
67
-
-
0032037476
-
Oncostatin M is a potent stimulator of alpha1-antitrypsin secretion in lung epithelial cells: Modulation by transforming growth factor-beta and interferon-gamma
-
4
-
Boutten A, Venembre P, Seta N, Hamelin J, Aubier M, Durand G, Dehoux MS (1998) Oncostatin M is a potent stimulator of alpha1-antitrypsin secretion in lung epithelial cells: modulation by transforming growth factor-beta and interferon-gamma. Am J Respir Cell Mol Biol 18(4):511-520
-
(1998)
Am J Respir Cell Mol Biol
, vol.18
, pp. 511-520
-
-
Boutten, A.1
Venembre, P.2
Seta, N.3
Hamelin, J.4
Aubier, M.5
Durand, G.6
Dehoux, M.S.7
-
68
-
-
0036547749
-
Gene regulation of the serine proteinase inhibitors alpha1-antitrypsin and alpha1-antichymotrypsin
-
2
-
Kalsheker N, Morley S, Morgan K (2002) Gene regulation of the serine proteinase inhibitors alpha1-antitrypsin and alpha1-antichymotrypsin. Biochem Soc Trans 30(2):93-98
-
(2002)
Biochem Soc Trans
, vol.30
, pp. 93-98
-
-
Kalsheker, N.1
Morley, S.2
Morgan, K.3
-
69
-
-
2442488539
-
Z alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant
-
5
-
Mulgrew AT, Taggart CC, Lawless MW, Greene CM, Brantly ML, O'Neill SJ, McElvaney NG (2004) Z alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant. Chest 125(5):1952-1957
-
(2004)
Chest
, vol.125
, pp. 1952-1957
-
-
Mulgrew, A.T.1
Taggart, C.C.2
Lawless, M.W.3
Greene, C.M.4
Brantly, M.L.5
O'Neill, S.J.6
McElvaney, N.G.7
-
70
-
-
0025360691
-
The protease-antiprotease balance within the human lung: Implications for the pathogenesis of emphysema
-
Gadek JE, Pacht ER (1990) The protease-antiprotease balance within the human lung: implications for the pathogenesis of emphysema. Lung 168 Suppl:552-564
-
(1990)
Lung 168
, pp. 552-564
-
-
Gadek, J.E.1
Pacht, E.R.2
-
71
-
-
0036014834
-
Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: A new paradigm for the pathogenesis of emphysema
-
6
-
Parmar JS, Mahadeva R, Reed BJ, Farahi N, Cadwallader KA, Keogan MT, Bilton D, Chilvers ER, Lomas DA (2002) Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema. Am J Respir Cell Mol Biol 26(6):723-730
-
(2002)
Am J Respir Cell Mol Biol
, vol.26
, pp. 723-730
-
-
Parmar, J.S.1
Mahadeva, R.2
Reed, B.J.3
Farahi, N.4
Cadwallader, K.A.5
Keogan, M.T.6
Bilton, D.7
Chilvers, E.R.8
Lomas, D.A.9
-
72
-
-
25944452730
-
Protease-complexed and polymerised antithrombin and alpha1-antitrypsin bind low density lipoprotein receptor-related protein
-
Suppl
-
Wardell MR, Lomas DA, Brecht WJ, Mahley RW (1994) Protease-complexed and polymerised antithrombin and alpha1-antitrypsin bind low density lipoprotein receptor-related protein. Protein Sci 3(Suppl):S101
-
(1994)
Protein Sci
, vol.3
, pp. 101
-
-
Wardell, M.R.1
Lomas, D.A.2
Brecht, W.J.3
Mahley, R.W.4
-
73
-
-
0023894870
-
The inhibitory complex of human alpha 1-proteinase inhibitor and human leukocyte elastase is a neutrophil chemoattractant
-
5
-
Banda MJ, Rice AG, Griffin GL, Senior RM (1988) The inhibitory complex of human alpha 1-proteinase inhibitor and human leukocyte elastase is a neutrophil chemoattractant. J Exp Med 167(5):1608-1615
-
(1988)
J Exp Med
, vol.167
, pp. 1608-1615
-
-
Banda, M.J.1
Rice, A.G.2
Griffin, G.L.3
Senior, R.M.4
-
74
-
-
0025614486
-
Acute phase protein stimulation by alpha 1-antichymotrypsin-cathepsin G complexes. Evidence for the involvement of interleukin-6
-
34
-
Kurdowska A, Travis J (1990) Acute phase protein stimulation by alpha 1-antichymotrypsin-cathepsin G complexes. Evidence for the involvement of interleukin-6. J Biol Chem 265(34):21023-21026
-
(1990)
J Biol Chem
, vol.265
, pp. 21023-21026
-
-
Kurdowska, A.1
Travis, J.2
-
75
-
-
19944430698
-
Polymers of Z alpha1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo
-
2
-
Mahadeva R, Atkinson C, Li Z, Stewart S, Janciauskiene S, Kelley DG, Parmar J, Pitman R, Shapiro SD, Lomas DA (2005) Polymers of Z alpha1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo. Am J Pathol 166(2):377-386
-
(2005)
Am J Pathol
, vol.166
, pp. 377-386
-
-
Mahadeva, R.1
Atkinson, C.2
Li, Z.3
Stewart, S.4
Janciauskiene, S.5
Kelley, D.G.6
Parmar, J.7
Pitman, R.8
Shapiro, S.D.9
Lomas, D.A.10
-
76
-
-
2142768895
-
Activation of endoplasmic reticulum-specific stress responses associated with the conformational disease Z alpha 1-antitrypsin deficiency
-
9
-
Lawless MW, Greene CM, Mulgrew A, Taggart CC, O'Neill SJ, McElvaney NG (2004) Activation of endoplasmic reticulum-specific stress responses associated with the conformational disease Z alpha 1-antitrypsin deficiency. J Immunol 172(9):5722-5726
-
(2004)
J Immunol
, vol.172
, pp. 5722-5726
-
-
Lawless, M.W.1
Greene, C.M.2
Mulgrew, A.3
Taggart, C.C.4
O'Neill, S.J.5
McElvaney, N.G.6
-
77
-
-
0029001576
-
A novel signal transduction pathway from the endoplasmic reticulum to the nucleus is mediated by transcription factor NF-kappa B
-
11
-
Pahl HL, Baeuerle PA (1995) A novel signal transduction pathway from the endoplasmic reticulum to the nucleus is mediated by transcription factor NF-kappa B. EMBO J 14(11):2580-2588
-
(1995)
EMBO J
, vol.14
, pp. 2580-2588
-
-
Pahl, H.L.1
Baeuerle, P.A.2
-
78
-
-
0033900004
-
A future for neonatal alpha1-antitrypsin screening?
-
6
-
Sveger T, Thelin T (2000) A future for neonatal alpha1-antitrypsin screening? Acta Paediatr 89(6):628-631
-
(2000)
Acta Paediatr
, vol.89
, pp. 628-631
-
-
Sveger, T.1
Thelin, T.2
-
79
-
-
0031035138
-
Young adults with alpha 1-antitrypsin deficiency identified neonatally: Their health, knowledge about and adaptation to the high-risk condition
-
1
-
Sveger T, Thelin T, McNeil TF (1997) Young adults with alpha 1-antitrypsin deficiency identified neonatally: their health, knowledge about and adaptation to the high-risk condition. Acta Paediatr 86(1):37-40
-
(1997)
Acta Paediatr
, vol.86
, pp. 37-40
-
-
Sveger, T.1
Thelin, T.2
McNeil, T.F.3
-
80
-
-
0035885239
-
Pilot detection study of alpha(1) antitrypsin deficiency in a targeted population
-
1
-
Fleming LE, Oquendo S, Bean JA, Tamer R, Finn S, Wanner A (2001) Pilot detection study of alpha(1) antitrypsin deficiency in a targeted population. Am J Med Genet 103(1):69-74
-
(2001)
Am J Med Genet
, vol.103
, pp. 69-74
-
-
Fleming, L.E.1
Oquendo, S.2
Bean, J.A.3
Tamer, R.4
Finn, S.5
Wanner, A.6
-
81
-
-
0035990476
-
Screening for alpha1-Pi deficiency in patients with lung diseases
-
2
-
Wencker M, Marx A, Konietzko N, Schaefer B, Campbell EJ (2002) Screening for alpha1-Pi deficiency in patients with lung diseases. Eur Respir J 20(2):319-324
-
(2002)
Eur Respir J
, vol.20
, pp. 319-324
-
-
Wencker, M.1
Marx, A.2
Konietzko, N.3
Schaefer, B.4
Campbell, E.J.5
-
82
-
-
0019787989
-
Replacement therapy of alpha 1-antitrypsin deficiency. Reversal of protease-antiprotease imbalance within the alveolar structures of PiZ subjects
-
5
-
Gadek JE, Klein HG, Holland PV, Crystal RG (1981) Replacement therapy of alpha 1-antitrypsin deficiency. Reversal of protease-antiprotease imbalance within the alveolar structures of PiZ subjects. J Clin Invest 68(5):1158-1165
-
(1981)
J Clin Invest
, vol.68
, pp. 1158-1165
-
-
Gadek, J.E.1
Klein, H.G.2
Holland, P.V.3
Crystal, R.G.4
-
83
-
-
0023148995
-
Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema
-
17
-
Wewers MD, Casalaro MA, Sellers SE, Swayze SC, McPhaul KM, Wittes JT, Crystal RG (1987) Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema. N Engl J Med 316(17):1055-1062
-
(1987)
N Engl J Med
, vol.316
, pp. 1055-1062
-
-
Wewers, M.D.1
Casalaro, M.A.2
Sellers, S.E.3
Swayze, S.C.4
McPhaul, K.M.5
Wittes, J.T.6
Crystal, R.G.7
-
84
-
-
0031242842
-
Does alpha1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha1-antitrypsin deficiency? Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL) alpha1-AT study group
-
10
-
Seersholm N, Wencker M, Banik N, Viskum K, Dirksen A, Kok-Jensen A, Konietzko N (1997) Does alpha1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha1-antitrypsin deficiency? Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL) alpha1-AT study group. Eur Respir J 10(10):2260-2263
-
(1997)
Eur Respir J
, vol.10
, pp. 2260-2263
-
-
Seersholm, N.1
Wencker, M.2
Banik, N.3
Viskum, K.4
Dirksen, A.5
Kok-Jensen, A.6
Konietzko, N.7
-
85
-
-
0037952677
-
Cost-effectiveness analysis of augmentation therapy for severe alpha1-antitrypsin deficiency
-
10
-
Gildea TR, Shermock KM, Singer ME, Stoller JK (2003) Cost-effectiveness analysis of augmentation therapy for severe alpha1-antitrypsin deficiency. Am J Respir Crit Care Med 167(10):1387-1392
-
(2003)
Am J Respir Crit Care Med
, vol.167
, pp. 1387-1392
-
-
Gildea, T.R.1
Shermock, K.M.2
Singer, M.E.3
Stoller, J.K.4
-
86
-
-
6344221451
-
Alpha1-antitrypsin deficiency. 6: New and emerging treatments for alpha1-antitrypsin deficiency
-
10
-
Sandhaus RA (2004) Alpha1-antitrypsin deficiency. 6: new and emerging treatments for alpha1-antitrypsin deficiency. Thorax 59(10):904-909
-
(2004)
Thorax
, vol.59
, pp. 904-909
-
-
Sandhaus, R.A.1
-
87
-
-
0024403417
-
Anti-neutrophil-elastase defenses of the lower respiratory tract in alpha 1-antitrypsin deficiency directly augmented with an aerosol of alpha 1-antitrypsin
-
3
-
Hubbard RC, Brantly ML, Sellers SE, Mitchell ME, Crystal RG (1989) Anti-neutrophil-elastase defenses of the lower respiratory tract in alpha 1-antitrypsin deficiency directly augmented with an aerosol of alpha 1-antitrypsin. Ann Intern Med 111(3):206-212
-
(1989)
Ann Intern Med
, vol.111
, pp. 206-212
-
-
Hubbard, R.C.1
Brantly, M.L.2
Sellers, S.E.3
Mitchell, M.E.4
Crystal, R.G.5
-
88
-
-
0024417082
-
Recombinant DNA-produced alpha 1-antitrypsin administered by aerosol augments lower respiratory tract antineutrophil elastase defenses in individuals with alpha 1-antitrypsin deficiency
-
4
-
Hubbard RC, McElvaney NG, Sellers SE, Healy JT, Czerski DB, Crystal RG (1989) Recombinant DNA-produced alpha 1-antitrypsin administered by aerosol augments lower respiratory tract antineutrophil elastase defenses in individuals with alpha 1-antitrypsin deficiency. J Clin Invest 84(4):1349-1354
-
(1989)
J Clin Invest
, vol.84
, pp. 1349-1354
-
-
Hubbard, R.C.1
McElvaney, N.G.2
Sellers, S.E.3
Healy, J.T.4
Czerski, D.B.5
Crystal, R.G.6
-
89
-
-
0024414888
-
Pulmonary deposition and clearance of aerosolized alpha-1-proteinase inhibitor administered to dogs and to sheep
-
4
-
Smith RM, Traber LD, Traber DL, Spragg RG (1989) Pulmonary deposition and clearance of aerosolized alpha-1-proteinase inhibitor administered to dogs and to sheep. J Clin Invest 84(4):1145-1154
-
(1989)
J Clin Invest
, vol.84
, pp. 1145-1154
-
-
Smith, R.M.1
Traber, L.D.2
Traber, D.L.3
Spragg, R.G.4
-
90
-
-
0023554087
-
The effect of a peptide aldehyde reversible inhibitor of elastase on a human leucocyte elastase-induced model of emphysema in the hamster
-
5
-
Kennedy AJ, Cline A, Ney UM, Johnson WH, Roberts NA (1987) The effect of a peptide aldehyde reversible inhibitor of elastase on a human leucocyte elastase-induced model of emphysema in the hamster. Eur J Respir Dis 71(5):472-478
-
(1987)
Eur J Respir Dis
, vol.71
, pp. 472-478
-
-
Kennedy, A.J.1
Cline, A.2
Ney, U.M.3
Johnson, W.H.4
Roberts, N.A.5
-
91
-
-
0024587631
-
Pharmacological profile of the substituted beta-lactam L-659,286: A member of a new class of human PMN elastase inhibitors
-
1
-
Bonney RJ, Ashe B, Maycock A, Dellea P, Hand K, Osinga D, Fletcher D, Mumford R, Davies P, Frankenfield D, et al. (1989) Pharmacological profile of the substituted beta-lactam L-659,286: a member of a new class of human PMN elastase inhibitors. J Cell Biochem 39(1):47-53
-
(1989)
J Cell Biochem
, vol.39
, pp. 47-53
-
-
Bonney, R.J.1
Ashe, B.2
Maycock, A.3
Dellea, P.4
Hand, K.5
Osinga, D.6
Fletcher, D.7
Mumford, R.8
Davies, P.9
Frankenfield, D.10
-
92
-
-
0026600147
-
Inhibition of human leukocyte elastase (HLE) by N-substituted peptidyl trifluoromethyl ketones
-
4
-
Skiles JW, Fuchs V, Miao C, Sorcek R, Grozinger KG, Mauldin SC, Vitous J, Mui PW, Jacober S, Chow G, et al. (1992) Inhibition of human leukocyte elastase (HLE) by N-substituted peptidyl trifluoromethyl ketones. J Med Chem 35(4):641-662
-
(1992)
J Med Chem
, vol.35
, pp. 641-662
-
-
Skiles, J.W.1
Fuchs, V.2
Miao, C.3
Sorcek, R.4
Grozinger, K.G.5
Mauldin, S.C.6
Vitous, J.7
Mui, P.W.8
Jacober, S.9
Chow, G.10
-
93
-
-
0018872475
-
Liver transplantation for advanced liver disease with alpha-1-antitrypsin deficiency
-
5
-
Hood JM, Koep LJ, Peters RL, Schroter GP, Weil R 3rd, Redeker AG, Starzl TE (1980) Liver transplantation for advanced liver disease with alpha-1-antitrypsin deficiency. N Engl J Med 302(5):272-275
-
(1980)
N Engl J Med
, vol.302
, pp. 272-275
-
-
Hood, J.M.1
Koep, L.J.2
Peters, R.L.3
Schroter, G.P.4
Weil III, R.5
Redeker, A.G.6
Starzl, T.E.7
-
94
-
-
0021215272
-
Orthotopic liver transplantation in children: Two-year experience with 47 patients
-
1
-
Gartner JC Jr, Zitelli BJ, Malatack JJ, Shaw BW, Iwatsuki S, Starzl TE (1984) Orthotopic liver transplantation in children: two-year experience with 47 patients. Pediatrics 74(1):140-145
-
(1984)
Pediatrics
, vol.74
, pp. 140-145
-
-
Gartner Jr., J.C.1
Zitelli, B.J.2
Malatack, J.J.3
Shaw, B.W.4
Iwatsuki, S.5
Starzl, T.E.6
-
95
-
-
0020578134
-
Reversal of hepatic alpha-1-antitrypsin deposition after portacaval shunt
-
8347
-
Starzl TE, Porter KA, Francavilla A, Iwatsuki S (1983) Reversal of hepatic alpha-1-antitrypsin deposition after portacaval shunt. Lancet 2(8347):424-426
-
(1983)
Lancet
, vol.2
, pp. 424-426
-
-
Starzl, T.E.1
Porter, K.A.2
Francavilla, A.3
Iwatsuki, S.4
-
96
-
-
0025155995
-
Portacaval shunt in three children with alpha-1-antitrypsin deficiency and cirrhosis: 9 to 12 1/3 years later
-
1
-
Starzl TE, Porter KA, Busuttil RW, Pichlmayr R (1990) Portacaval shunt in three children with alpha-1-antitrypsin deficiency and cirrhosis: 9 to 12 1/3 years later. Hepatology 11(1):152-154
-
(1990)
Hepatology
, vol.11
, pp. 152-154
-
-
Starzl, T.E.1
Porter, K.A.2
Busuttil, R.W.3
Pichlmayr, R.4
-
97
-
-
0025815961
-
Alpha 1-antitrypsin deficiency and liver disease: Clinical presentation, diagnosis and treatment
-
4
-
Hussain M, Mieli-Vergani G, Mowat AP (1991) Alpha 1-antitrypsin deficiency and liver disease: clinical presentation, diagnosis and treatment. J Inherit Metab Dis 14(4):497-511
-
(1991)
J Inherit Metab Dis
, vol.14
, pp. 497-511
-
-
Hussain, M.1
Mieli-Vergani, G.2
Mowat, A.P.3
-
98
-
-
0026691478
-
Cell migration, chimerism, and graft acceptance
-
8809
-
Starzl TE, Demetris AJ, Murase N, Ildstad S, Ricordi C, Trucco M (1992) Cell migration, chimerism, and graft acceptance. Lancet 339(8809):1579-1582
-
(1992)
Lancet
, vol.339
, pp. 1579-1582
-
-
Starzl, T.E.1
Demetris, A.J.2
Murase, N.3
Ildstad, S.4
Ricordi, C.5
Trucco, M.6
-
99
-
-
0023197990
-
Clonal gene therapy: Transplanted mouse fibroblast clones express human alpha 1-antitrypsin gene in vivo
-
4816
-
Garver RI Jr, Chytil A, Courtney M, Crystal RG (1987) Clonal gene therapy: transplanted mouse fibroblast clones express human alpha 1-antitrypsin gene in vivo. Science 237(4816):762-764
-
(1987)
Science
, vol.237
, pp. 762-764
-
-
Garver Jr., R.I.1
Chytil, A.2
Courtney, M.3
Crystal, R.G.4
-
100
-
-
0027748178
-
Hepatic gene therapy: Efficient retroviral-mediated gene transfer into rat hepatocytes in vivo
-
5
-
Kolodka TM, Finegold M, Kay MA, Woo SL (1993) Hepatic gene therapy: efficient retroviral-mediated gene transfer into rat hepatocytes in vivo. Somat Cell Mol Genet 19(5):491-497
-
(1993)
Somat Cell Mol Genet
, vol.19
, pp. 491-497
-
-
Kolodka, T.M.1
Finegold, M.2
Kay, M.A.3
Woo, S.L.4
-
101
-
-
0025848772
-
Adenovirus-mediated transfer of a recombinant alpha 1-antitrypsin gene to the lung epithelium in vivo
-
5004
-
Rosenfeld MA, Siegfried W, Yoshimura K, Toneyama K, Fukayama M, Stier LE, Paakko PK, Gilardi P, Stratford-Perricaudet LD, Perricaudet M, et al. (1991) Adenovirus-mediated transfer of a recombinant alpha 1-antitrypsin gene to the lung epithelium in vivo. Science 252(5004):431-434
-
(1991)
Science
, vol.252
, pp. 431-434
-
-
Rosenfeld, M.A.1
Siegfried, W.2
Yoshimura, K.3
Toneyama, K.4
Fukayama, M.5
Stier, L.E.6
Paakko, P.K.7
Gilardi, P.8
Stratford-Perricaudet, L.D.9
Perricaudet, M.10
-
102
-
-
0028060363
-
Efficient gene transfer into nondividing cells by adeno-associated virus-based vectors
-
9
-
Podsakoff G, Wong KK Jr, Chatterjee S (1994) Efficient gene transfer into nondividing cells by adeno-associated virus-based vectors. J Virol 68(9):5656-5666
-
(1994)
J Virol
, vol.68
, pp. 5656-5666
-
-
Podsakoff, G.1
Wong Jr., K.K.2
Chatterjee, S.3
-
103
-
-
0027399681
-
Adeno-associated virus: Integration at a specific chromosomal locus
-
1
-
Samulski RJ (1993) Adeno-associated virus: integration at a specific chromosomal locus. Curr Opin Genet Dev 3(1):74-80
-
(1993)
Curr Opin Genet Dev
, vol.3
, pp. 74-80
-
-
Samulski, R.J.1
-
105
-
-
13144305062
-
Sustained secretion of human alpha-1-antitrypsin from murine muscle transduced with adeno-associated virus vectors
-
24
-
Song S, Morgan M, Ellis T, Poirier A, Chesnut K, Wang J, Brantly M, Muzyczka N, Byrne BJ, Atkinson M, Flotte TR (1998) Sustained secretion of human alpha-1-antitrypsin from murine muscle transduced with adeno-associated virus vectors. Proc Natl Acad Sci U S A 95(24):14384-14388
-
(1998)
Proc Natl Acad Sci U S a
, vol.95
, pp. 14384-14388
-
-
Song, S.1
Morgan, M.2
Ellis, T.3
Poirier, A.4
Chesnut, K.5
Wang, J.6
Brantly, M.7
Muzyczka, N.8
Byrne, B.J.9
Atkinson, M.10
Flotte, T.R.11
-
106
-
-
0742307402
-
Phase I trial of intramuscular injection of a recombinant adeno-associated virus alpha 1-antitrypsin (rAAV2-CB-hAAT) gene vector to AAT-deficient adults
-
1
-
Flotte TR, Brantly ML, Spencer LT, Byrne BJ, Spencer CT, Baker DJ, Humphries M (2004) Phase I trial of intramuscular injection of a recombinant adeno-associated virus alpha 1-antitrypsin (rAAV2-CB-hAAT) gene vector to AAT-deficient adults. Hum Gene Ther 15(1):93-128
-
(2004)
Hum Gene Ther
, vol.15
, pp. 93-128
-
-
Flotte, T.R.1
Brantly, M.L.2
Spencer, L.T.3
Byrne, B.J.4
Spencer, C.T.5
Baker, D.J.6
Humphries, M.7
-
107
-
-
0032886295
-
Rationale and design of the National Emphysema Treatment Trial (NETT): A prospective randomized trial of lung volume reduction surgery
-
No authors listed
-
No authors listed (1999) Rationale and design of the National Emphysema Treatment Trial (NETT): A prospective randomized trial of lung volume reduction surgery. J Thorac Cardiovasc Surg 118(3):518-528
-
(1999)
J Thorac Cardiovasc Surg
, vol.118
, Issue.3
, pp. 518-528
-
-
-
108
-
-
0036774085
-
Evidence-based medicine: Lung volume reduction surgery (LVRS)
-
5
-
Koebe HG, Kugler C, Dienemann H (2002) Evidence-based medicine: lung volume reduction surgery (LVRS). Thorac Cardiovasc Surg 50(5):315-322
-
(2002)
Thorac Cardiovasc Surg
, vol.50
, pp. 315-322
-
-
Koebe, H.G.1
Kugler, C.2
Dienemann, H.3
-
109
-
-
4444268460
-
Long-term results after lung volume reduction surgery in patients with alpha1-antitrypsin deficiency
-
3
-
Tutic M, Bloch KE, Lardinois D, Brack T, Russi EW, Weder W (2004) Long-term results after lung volume reduction surgery in patients with alpha1-antitrypsin deficiency. J Thorac Cardiovasc Surg 128(3):408-413
-
(2004)
J Thorac Cardiovasc Surg
, vol.128
, pp. 408-413
-
-
Tutic, M.1
Bloch, K.E.2
Lardinois, D.3
Brack, T.4
Russi, E.W.5
Weder, W.6
-
110
-
-
0024468467
-
Unilateral lung transplantation in end-stage pulmonary emphysema
-
3
-
Mal H, Andreassian B, Pamela F, Duchatelle JP, Rondeau E, Dubois F, Baldeyrou P, Kitzis M, Sleiman C, Pariente R (1989) Unilateral lung transplantation in end-stage pulmonary emphysema. Am Rev Respir Dis 140(3):797-802
-
(1989)
Am Rev Respir Dis
, vol.140
, pp. 797-802
-
-
Mal, H.1
Andreassian, B.2
Pamela, F.3
Duchatelle, J.P.4
Rondeau, E.5
Dubois, F.6
Baldeyrou, P.7
Kitzis, M.8
Sleiman, C.9
Pariente, R.10
-
111
-
-
0026697229
-
Morbidity, mortality, and early results of single versus bilateral lung transplantation for emphysema
-
6
-
Low DE, Trulock EP, Kaiser LR, Pasque MK, Dresler C, Ettinger N, Cooper JD (1992) Morbidity, mortality, and early results of single versus bilateral lung transplantation for emphysema. J Thorac Cardiovasc Surg 103(6):1119-1126
-
(1992)
J Thorac Cardiovasc Surg
, vol.103
, pp. 1119-1126
-
-
Low, D.E.1
Trulock, E.P.2
Kaiser, L.R.3
Pasque, M.K.4
Dresler, C.5
Ettinger, N.6
Cooper, J.D.7
-
112
-
-
0036839848
-
Thirteen-year experience in lung transplantation for emphysema
-
5. discussion 1669-1670
-
Cassivi SD, Meyers BF, Barrafarano RJ, Guthrie TJ, Trulock EP, Lynch JP, Cooer JD, Patterson GA (2002) Thirteen-year experience in lung transplantation for emphysema. Ann Thorac Surg 74(5):1663-1669; discussion 1669-1670
-
(2002)
Ann Thorac Surg
, vol.74
, pp. 1663-1669
-
-
Cassivi, S.D.1
Meyers, B.F.2
Barrafarano, R.J.3
Guthrie, T.J.4
Trulock, E.P.5
Lynch, J.P.6
Cooer, J.D.7
Patterson, G.A.8
-
113
-
-
0028351634
-
The proteinase-antiproteinase balance in alpha-1-proteinase inhibitor-deficient lung transplant recipients
-
4 Pt 1
-
King MB, Campbell EJ, Gray BH, Hertz MI (1994) The proteinase- antiproteinase balance in alpha-1-proteinase inhibitor-deficient lung transplant recipients. Am J Respir Crit Care Med 149(4 Pt 1):966-971
-
(1994)
Am J Respir Crit Care Med
, vol.149
, pp. 966-971
-
-
King, M.B.1
Campbell, E.J.2
Gray, B.H.3
Hertz, M.I.4
-
114
-
-
0032110436
-
Survival and FEV1 decline in individuals with severe deficiency of alpha1-antitrypsin
-
No authors listed The Alpha-1-Antitrypsin Deficiency Registry Study Group.
-
No authors listed (1998) Survival and FEV1 decline in individuals with severe deficiency of alpha1-antitrypsin. The Alpha-1-Antitrypsin Deficiency Registry Study Group. Am J Respir Crit Care Med 158(1):49-59
-
(1998)
Am J Respir Crit Care Med
, vol.158
, Issue.1
, pp. 49-59
-
-
-
115
-
-
0015847261
-
1-Antitrypsin deficiency: A variant with no detectable 1 -antitrypsin
-
94
-
Talamo RC, Langley CE, Reed CE, Makino S (1973) 1-Antitrypsin deficiency: a variant with no detectable 1 -antitrypsin. Science 181(94):70-71
-
(1973)
Science
, vol.181
, pp. 70-71
-
-
Talamo, R.C.1
Langley, C.E.2
Reed, C.E.3
Makino, S.4
-
116
-
-
0022633717
-
Alpha 1-antitrypsin deficiency and emphysema caused by homozygous inheritance of non-expressing alpha 1-antitrypsin genes
-
12
-
Garver RI Jr, Mornex JF, Nukiwa T, Brantly M, Courtney M, LeCocq JP, Crystal RG (1986) Alpha 1-antitrypsin deficiency and emphysema caused by homozygous inheritance of non-expressing alpha 1-antitrypsin genes. N Engl J Med 314(12):762-766
-
(1986)
N Engl J Med
, vol.314
, pp. 762-766
-
-
Garver Jr., R.I.1
Mornex, J.F.2
Nukiwa, T.3
Brantly, M.4
Courtney, M.5
Lecocq, J.P.6
Crystal, R.G.7
-
117
-
-
0036606880
-
The effect of augmentation therapy on bronchial inflammation in alpha1-antitrypsin deficiency
-
11
-
Stockley RA, Bayley DL, Unsal I, Dowson LJ (2002) The effect of augmentation therapy on bronchial inflammation in alpha1-antitrypsin deficiency. Am J Respir Crit Care Med 165(11):1494-1498
-
(2002)
Am J Respir Crit Care Med
, vol.165
, pp. 1494-1498
-
-
Stockley, R.A.1
Bayley, D.L.2
Unsal, I.3
Dowson, L.J.4
-
118
-
-
4143133190
-
Inhibiting polymerization: New therapeutic strategies for Z alpha1-antitrypsin-related emphysema
-
2
-
Parfrey H, Dafforn TR, Belorgey D, Lomas DA, Mahadeva R (2004) Inhibiting polymerization: new therapeutic strategies for Z alpha1-antitrypsin-related emphysema. Am J Respir Cell Mol Biol 31(2):133-139
-
(2004)
Am J Respir Cell Mol Biol
, vol.31
, pp. 133-139
-
-
Parfrey, H.1
Dafforn, T.R.2
Belorgey, D.3
Lomas, D.A.4
Mahadeva, R.5
-
119
-
-
0036510604
-
6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization. Implications for the prevention of Z alpha(1)-antitrypsin-related cirrhosis
-
9
-
Mahadeva R, Dafforn TR, Carrell RW, Lomas DA (2002) 6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization. Implications for the prevention of Z alpha(1)-antitrypsin- related cirrhosis. J Biol Chem 277(9):6771-6774
-
(2002)
J Biol Chem
, vol.277
, pp. 6771-6774
-
-
Mahadeva, R.1
Dafforn, T.R.2
Carrell, R.W.3
Lomas, D.A.4
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