-
1
-
-
79551604651
-
Alpha-1-antitrypsin deficiency: importance of proteasomal and autophagic degradative pathways in disposal of liver disease-associated protein aggregates
-
Perlmutter DH. Alpha-1-antitrypsin deficiency: importance of proteasomal and autophagic degradative pathways in disposal of liver disease-associated protein aggregates. Annu Rev Med 2011;62:333-345.
-
(2011)
Annu Rev Med
, vol.62
, pp. 333-345
-
-
Perlmutter, D.H.1
-
3
-
-
70349151807
-
Burden and pathogenesis of chronic obstructive pulmonary disease
-
Stockley RA, Mannino D, Barnes PJ. Burden and pathogenesis of chronic obstructive pulmonary disease. Proc Am Thorac Soc 2009;6:524-526.
-
(2009)
Proc Am Thorac Soc
, vol.6
, pp. 524-526
-
-
Stockley, R.A.1
Mannino, D.2
Barnes, P.J.3
-
4
-
-
0014530551
-
Cirrhosis associated with alpha-1-antitrypsin deficiency: a previously unrecognized inherited disorder
-
Sharp HL, Bridges RA, Krivit W, Freier EF. Cirrhosis associated with alpha-1-antitrypsin deficiency: a previously unrecognized inherited disorder. J Lab Clin Med 1969;73:934-939.
-
(1969)
J Lab Clin Med
, vol.73
, pp. 934-939
-
-
Sharp, H.L.1
Bridges, R.A.2
Krivit, W.3
Freier, E.F.4
-
5
-
-
0033623281
-
Alpha1-antitrypsin deficiency-associated liver disease progresses slowly in some children
-
Volpert D, Molleston JP, Perlmutter DH. Alpha1-antitrypsin deficiency-associated liver disease progresses slowly in some children. J Pediatr Gastroenterol Nutr 2000;31:258-263.
-
(2000)
J Pediatr Gastroenterol Nutr
, vol.31
, pp. 258-263
-
-
Volpert, D.1
Molleston, J.P.2
Perlmutter, D.H.3
-
6
-
-
84897940985
-
Mysteries of α1-antitrypsin deficiency: emerging therapeutic strategies for a challenging disease
-
Ghouse R, Chu A, Wang Y, Perlmutter DH. Mysteries of α1-antitrypsin deficiency: emerging therapeutic strategies for a challenging disease. Dis Model Mech 2014;7:411-419.
-
(2014)
Dis Model Mech
, vol.7
, pp. 411-419
-
-
Ghouse, R.1
Chu, A.2
Wang, Y.3
Perlmutter, D.H.4
-
7
-
-
33748062353
-
Pathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency
-
Perlmutter DH. Pathogenesis of chronic liver injury and hepatocellular carcinoma in alpha-1-antitrypsin deficiency. Pediatr Res 2006;60:233-238.
-
(2006)
Pediatr Res
, vol.60
, pp. 233-238
-
-
Perlmutter, D.H.1
-
8
-
-
0022637178
-
Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency
-
Eriksson S, Carlson J, Velez R. Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency. N Engl J Med 1986;314:736-739.
-
(1986)
N Engl J Med
, vol.314
, pp. 736-739
-
-
Eriksson, S.1
Carlson, J.2
Velez, R.3
-
9
-
-
0020517872
-
Alpha-1-antitrypsin MZ phenotype and cryptogenic chronic liver disease in adults
-
Vecchio FM, Fabiano A, Orsini G, Ragusa D, Massi G. Alpha-1-antitrypsin MZ phenotype and cryptogenic chronic liver disease in adults. Digestion 1983;27:100-104.
-
(1983)
Digestion
, vol.27
, pp. 100-104
-
-
Vecchio, F.M.1
Fabiano, A.2
Orsini, G.3
Ragusa, D.4
Massi, G.5
-
10
-
-
34547440421
-
Heterozygous alpha-I antitrypsin deficiency as a co-factor in the development of chronic liver disease: a review
-
Kok KF, Wahab PJ, Houwen RH, Drenth JP, de Man RA, van Hoek B, et al. Heterozygous alpha-I antitrypsin deficiency as a co-factor in the development of chronic liver disease: a review. Neth J Med 2007;65:160-166.
-
(2007)
Neth J Med
, vol.65
, pp. 160-166
-
-
Kok, K.F.1
Wahab, P.J.2
Houwen, R.H.3
Drenth, J.P.4
de Man, R.A.5
van Hoek, B.6
-
11
-
-
0019449013
-
Heterozygous MZ alpha1-antitrypsin deficiency in adults with chronic active hepatitis and cryptogenic cirrhosis
-
Hodges JR, Millward-Sadler GH, Barbatis C, Wright R. Heterozygous MZ alpha1-antitrypsin deficiency in adults with chronic active hepatitis and cryptogenic cirrhosis. N Engl J Med 1981;304:557-560.
-
(1981)
N Engl J Med
, vol.304
, pp. 557-560
-
-
Hodges, J.R.1
Millward-Sadler, G.H.2
Barbatis, C.3
Wright, R.4
-
12
-
-
0025289619
-
Heterozygous MZ alpha-1-antitrypsin deficiency in adults with chronic liver disease
-
Bell H, Schrumpf E, Fagerhol MK. Heterozygous MZ alpha-1-antitrypsin deficiency in adults with chronic liver disease. Scand J Gastroenterol 1990;25:788-792.
-
(1990)
Scand J Gastroenterol
, vol.25
, pp. 788-792
-
-
Bell, H.1
Schrumpf, E.2
Fagerhol, M.K.3
-
13
-
-
33846036488
-
High prevalence of alpha-1-antitrypsin heterozygosity in children with chronic liver disease
-
Campbell KM, Arya G, Ryckman FC, Alonso M, Tiao G, Balistreri WF, Bezerra JA. High prevalence of alpha-1-antitrypsin heterozygosity in children with chronic liver disease. J Pediatr Gastroenterol Nutr 2007;44:99-103.
-
(2007)
J Pediatr Gastroenterol Nutr
, vol.44
, pp. 99-103
-
-
Campbell, K.M.1
Arya, G.2
Ryckman, F.C.3
Alonso, M.4
Tiao, G.5
Balistreri, W.F.6
Bezerra, J.A.7
-
14
-
-
0031682958
-
Increased risk of chronic liver failure in adults with heterozygous alpha1-antitrypsin deficiency
-
Graziadei IW, Joseph JJ, Wiesner RH, Therneau TM, Batts KP, Porayko MK. Increased risk of chronic liver failure in adults with heterozygous alpha1-antitrypsin deficiency. Hepatology 1998;28:1058-1063.
-
(1998)
Hepatology
, vol.28
, pp. 1058-1063
-
-
Graziadei, I.W.1
Joseph, J.J.2
Wiesner, R.H.3
Therneau, T.M.4
Batts, K.P.5
Porayko, M.K.6
-
15
-
-
0021172570
-
Alpha-1-antitrypsin deficiency
-
Alagille D. Alpha-1-antitrypsin deficiency. Hepatology 1984;4(suppl):11S-14S.
-
(1984)
Hepatology
, vol.4
, pp. 11S-14S
-
-
Alagille, D.1
-
16
-
-
0017099344
-
Liver disease in alpha1-antitrypsin deficiency detected by screening of 200,000 infants
-
Sveger T. Liver disease in alpha1-antitrypsin deficiency detected by screening of 200,000 infants. N Engl J Med 1976;294:1316-1321.
-
(1976)
N Engl J Med
, vol.294
, pp. 1316-1321
-
-
Sveger, T.1
-
17
-
-
27144481559
-
Alpha1-antitrypsin deficiency in 26-year-old subjects: lung, liver, and protease/protease inhibitor studies
-
Piitulainen E, Carlson J, Ohlsson K, Sveger T. Alpha1-antitrypsin deficiency in 26-year-old subjects: lung, liver, and protease/protease inhibitor studies. Chest 2005;128:2076-2081.
-
(2005)
Chest
, vol.128
, pp. 2076-2081
-
-
Piitulainen, E.1
Carlson, J.2
Ohlsson, K.3
Sveger, T.4
-
18
-
-
77954597127
-
An autophagy-enhancing drug promotes degradation of mutant alpha1-antitrypsin Z and reduces hepatic fibrosis
-
Hidvegi T, Ewing M, Hale P, Dippold C, Beckett C, Kemp C, et al. An autophagy-enhancing drug promotes degradation of mutant alpha1-antitrypsin Z and reduces hepatic fibrosis. Science 2010;329:229-232.
-
(2010)
Science
, vol.329
, pp. 229-232
-
-
Hidvegi, T.1
Ewing, M.2
Hale, P.3
Dippold, C.4
Beckett, C.5
Kemp, C.6
-
20
-
-
1842532017
-
Analyses of hepatocellular proliferation in a mouse model of alpha-1-antitrypsin deficiency
-
Rudnick DA, Liao Y, An JK, Muglia LJ, Perlmutter DH, Teckman JH. Analyses of hepatocellular proliferation in a mouse model of alpha-1-antitrypsin deficiency. Hepatology 2004;39:1048-1055.
-
(2004)
Hepatology
, vol.39
, pp. 1048-1055
-
-
Rudnick, D.A.1
Liao, Y.2
An, J.K.3
Muglia, L.J.4
Perlmutter, D.H.5
Teckman, J.H.6
-
21
-
-
0018875422
-
Danazol-induced augmentation of serum α1-antitrypsin levels in individuals with marked deficiency of this antiprotease
-
Gadek JE, Fulmer JD, Gelfand JA, Frank MM, Petty TL, Crystal RG. Danazol-induced augmentation of serum α1-antitrypsin levels in individuals with marked deficiency of this antiprotease. J Clin Invest 1980;66:82-87.
-
(1980)
J Clin Invest
, vol.66
, pp. 82-87
-
-
Gadek, J.E.1
Fulmer, J.D.2
Gelfand, J.A.3
Frank, M.M.4
Petty, T.L.5
Crystal, R.G.6
|