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Volumn 8, Issue 1, 2006, Pages 14-20

Alpha-1-antitrypsin deficiency: Diagnosis, pathophysiology, and management

Author keywords

[No Author keywords available]

Indexed keywords

4 PHENYLBUTYRIC ACID; ALANINE AMINOTRANSFERASE; ALPHA 1 ANTITRYPSIN; ASPARTATE AMINOTRANSFERASE; CASPASE;

EID: 33645079400     PISSN: 15228037     EISSN: None     Source Type: Journal    
DOI: 10.1007/s11894-006-0059-8     Document Type: Review
Times cited : (36)

References (52)
  • 1
    • 4744356921 scopus 로고    scopus 로고
    • Alpha-1-antitrypsin deficiency: Diagnosis and treatment
    • viii-ix
    • Perlmutter DH: Alpha-1-antitrypsin deficiency: diagnosis and treatment. Clin Liver Dis 2004, 8:839-859, viii-ix.
    • (2004) Clin Liver Dis , vol.8 , pp. 839-859
    • Perlmutter, D.H.1
  • 2
    • 0141706635 scopus 로고    scopus 로고
    • Standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency
    • American Thoracic Society/European Respiratory Society Statement
    • 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.
    • (2003) Am J Respir Crit Care Med , vol.168 , pp. 818-900
  • 3
    • 2642680855 scopus 로고    scopus 로고
    • Alpha 1-antitrypsin deficiency: Memorandum from a WHO meeting
    • Alpha 1-antitrypsin deficiency: memorandum from a WHO meeting. Bull World Health Organ 1997, 75:397-415.
    • (1997) Bull World Health Organ , vol.75 , pp. 397-415
  • 4
    • 0017099344 scopus 로고
    • 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
  • 5
    • 0017888482 scopus 로고
    • alpha 1-antitrypsin deficiency in early childhood
    • Sveger T: alpha 1-antitrypsin deficiency in early childhood. Pediatrics 1978, 62:22-25.
    • (1978) Pediatrics , vol.62 , pp. 22-25
    • Sveger, T.1
  • 6
    • 0028152940 scopus 로고
    • Lung function in adolescents with alpha 1-antitrypsin deficiency
    • Sveger T, Piitulainen E, Arborelius M, Jr: Lung function in adolescents with alpha 1-antitrypsin deficiency. Acta Paediatr 1994, 83:1170-1173.
    • (1994) Acta Paediatr , vol.83 , pp. 1170-1173
    • Sveger, T.1    Piitulainen, E.2    Arborelius Jr., M.3
  • 7
    • 0028236172 scopus 로고
    • Alpha 1-antitrypsin deficiency (PiZZ): Features of liver involvement in childhood
    • Mowat AP: Alpha 1-antitrypsin deficiency (PiZZ): features of liver involvement in childhood. Acta Paediatr Suppl 1994, 393:13-7.
    • (1994) Acta Paediatr Suppl , vol.393 , pp. 13-17
    • Mowat, A.P.1
  • 8
    • 0024266978 scopus 로고
    • Alpha 1 antitrypsin deficiency in two population groups in north Italy
    • Pittschieler K, Massi C: Alpha 1 antitrypsin deficiency in two population groups in north Italy. Padiatr Padol 1988, 23:307-311.
    • (1988) Padiatr Padol , vol.23 , pp. 307-311
    • Pittschieler, K.1    Massi, C.2
  • 9
    • 0029083915 scopus 로고
    • The liver in adolescents with alpha 1-antitrypsin deficiency
    • Sveger T, Eriksson S: The liver in adolescents with alpha 1-antitrypsin deficiency. Hepatology 1995, 22:514-517.
    • (1995) Hepatology , vol.22 , pp. 514-517
    • Sveger, T.1    Eriksson, S.2
  • 10
    • 33645056755 scopus 로고    scopus 로고
    • Alpha-1-antitrypsin deficiency: Natural course and therapeutic strategies
    • Edited by Boyer JL, Blum HE, Maier HE, et al. Dordrecht: Kluwer Academic Publishers
    • Eriksson S: Alpha-1-antitrypsin deficiency: natural course and therapeutic strategies. In Falk Symposium 115: Liver Cirrhosis and Its Development. Edited by Boyer JL, Blum HE, Maier HE, et al. Dordrecht: Kluwer Academic Publishers; 2001:307-315.
    • (2001) Falk Symposium 115: Liver Cirrhosis and Its Development , pp. 307-315
    • Eriksson, S.1
  • 11
    • 0032910916 scopus 로고    scopus 로고
    • Alpha 1-antitrypsin deficiency
    • Eriksson S: Alpha 1-antitrypsin deficiency. J Hepatol 1999, 30(Suppl 1):34-39.
    • (1999) J Hepatol , vol.30 , Issue.SUPPL. 1 , pp. 34-39
    • Eriksson, S.1
  • 12
    • 11244346111 scopus 로고    scopus 로고
    • Quantitative isolation of alpha1AT mutant Z protein polymers from human and mouse livers and the effect of heat
    • An JK, Blomenkamp K, Lindblad D, Teckman JH: Quantitative isolation of alpha1AT mutant Z protein polymers from human and mouse livers and the effect of heat. Hepatology 2005, 41:160-167.
    • (2005) Hepatology , vol.41 , pp. 160-167
    • An, J.K.1    Blomenkamp, K.2    Lindblad, D.3    Teckman, J.H.4
  • 13
    • 0029794112 scopus 로고    scopus 로고
    • Alpha-1-antitrypsin deficiency: Biochemistry and clinical manifestations
    • Perlmutter DH: Alpha-1-antitrypsin deficiency: biochemistry and clinical manifestations. Ann Med 1996, 28:385-394.
    • (1996) Ann Med , vol.28 , pp. 385-394
    • Perlmutter, D.H.1
  • 14
    • 0028593988 scopus 로고
    • A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency
    • Wu Y, Whitman I, Molmenti E, et al.: 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 1994, 91:9014-9018.
    • (1994) Proc Natl Acad Sci U S A , vol.91 , pp. 9014-9018
    • Wu, Y.1    Whitman, I.2    Molmenti, E.3
  • 15
    • 0029788023 scopus 로고    scopus 로고
    • Degradation of a mutant secretory protein, alpha1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity
    • Qu D, Teckman JH, Omura S, Perlmutter DH: Degradation of a mutant secretory protein, alpha1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity. J Biol Chem 1996, 271:22791-22795.
    • (1996) J Biol Chem , vol.271 , pp. 22791-22795
    • Qu, D.1    Teckman, J.H.2    Omura, S.3    Perlmutter, D.H.4
  • 16
    • 2942638034 scopus 로고    scopus 로고
    • Alpha1-antitrypsin deficiency. 4: Molecular pathophysiology
    • Lomas DA, Parfrey H: Alpha1-antitrypsin deficiency. 4: Molecular pathophysiology. Thorax 2004, 59:529-535.
    • (2004) Thorax , vol.59 , pp. 529-535
    • Lomas, D.A.1    Parfrey, H.2
  • 17
    • 0033515559 scopus 로고    scopus 로고
    • A kinetic mechanism for the polymerization of alpha1-antitrypsin
    • Dafforn TR, Mahadeva R, Elliott PR, et al.: A kinetic mechanism for the polymerization of alpha1-antitrypsin. J Biol Chem 1999, 274:9548-9555.
    • (1999) J Biol Chem , vol.274 , pp. 9548-9555
    • Dafforn, T.R.1    Mahadeva, R.2    Elliott, P.R.3
  • 18
    • 0026755363 scopus 로고
    • The mechanism of Z alpha 1-antitrypsin accumulation in the liver
    • Lomas DA, Evans DL, Finch JT, Carrell RW: The mechanism of Z alpha 1-antitrypsin accumulation in the liver. Nature 1992, 357:605-607.
    • (1992) Nature , vol.357 , pp. 605-607
    • Lomas, D.A.1    Evans, D.L.2    Finch, J.T.3    Carrell, R.W.4
  • 19
    • 0034637555 scopus 로고    scopus 로고
    • Processing by endoplasmic reticulum mannosidases partitions a secretion-impaired glycoprotein into distinct disposal pathways
    • Cabral CM, Choudhury P, Liu Y, Sifers RN: Processing by endoplasmic reticulum mannosidases partitions a secretion-impaired glycoprotein into distinct disposal pathways. J Biol Chem 2000, 275:25015-25022.
    • (2000) J Biol Chem , vol.275 , pp. 25015-25022
    • Cabral, C.M.1    Choudhury, P.2    Liu, Y.3    Sifers, R.N.4
  • 20
    • 0035976919 scopus 로고    scopus 로고
    • The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes
    • Teckman JH, Burrows J, Hidvegi T, et al.: The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes. J Biol Chem 2001, 276: 44865-44872.
    • (2001) J Biol Chem , vol.276 , pp. 44865-44872
    • Teckman, J.H.1    Burrows, J.2    Hidvegi, T.3
  • 21
    • 0033671965 scopus 로고    scopus 로고
    • Retention of mutant alpha(1)-antitrypsin Z in endoplasmic reticulum is associated with an autophagic response
    • Teckman JH, Perlmutter DH: Retention of mutant alpha(1)-antitrypsin Z in endoplasmic reticulum is associated with an autophagic response. Am J Physiol Gastrointest Liver Physiol 2000, 279:G961-G974.
    • (2000) Am J Physiol Gastrointest Liver Physiol , vol.279
    • Teckman, J.H.1    Perlmutter, D.H.2
  • 22
    • 0037470326 scopus 로고    scopus 로고
    • Cell biology. Protein degradation unlocked
    • Sifers RN: Cell biology. Protein degradation unlocked. Science 2003, 299:1330-1331.
    • (2003) Science , vol.299 , pp. 1330-1331
    • Sifers, R.N.1
  • 23
  • 24
    • 1542466459 scopus 로고    scopus 로고
    • Alpha1-antitrypsin deficiency: Liver disease associated with retention of a mutant secretory glycoprotein in the endoplasmic reticulum
    • Perlmutter DH: Alpha1-antitrypsin deficiency: liver disease associated with retention of a mutant secretory glycoprotein in the endoplasmic reticulum. Methods Mol Biol 2003, 232:39-56.
    • (2003) Methods Mol Biol , vol.232 , pp. 39-56
    • Perlmutter, D.H.1
  • 25
    • 0027473016 scopus 로고
    • Effect of the Z mutation on the physical and inhibitory properties of alpha 1-antitrypsin
    • Lomas DA, Evans DL, Stone SR. et al.: Effect of the Z mutation on the physical and inhibitory properties of alpha 1-antitrypsin. Biochemistry 1993, 32:500-508.
    • (1993) Biochemistry , vol.32 , pp. 500-508
    • Lomas, D.A.1    Evans, D.L.2    Stone, S.R.3
  • 26
    • 85047684827 scopus 로고    scopus 로고
    • Alpha1-antitrypsin polymerization and the serpinopathies: Pathobiology and prospects for therapy
    • Lomas DA, Mahadeva R: Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy. J Clin Invest 2002, 110:1585-1590.
    • (2002) J Clin Invest , vol.110 , pp. 1585-1590
    • Lomas, D.A.1    Mahadeva, R.2
  • 27
    • 0035823544 scopus 로고    scopus 로고
    • A naturally occurring nonpolymerogenic mutant of alpha 1-antitrypsin characterized by prolonged retention in the endoplasmic reticulum
    • Lin L, Schmidt B, Teckman J, Perlmutter DH: A naturally occurring nonpolymerogenic mutant of alpha 1-antitrypsin characterized by prolonged retention in the endoplasmic reticulum. J Biol Chem 2001, 276:33893-3898.
    • (2001) J Biol Chem , vol.276 , pp. 33893-33898
    • Lin, L.1    Schmidt, B.2    Teckman, J.3    Perlmutter, D.H.4
  • 28
    • 0037011795 scopus 로고    scopus 로고
    • Alpha1-antitrypsin deficiency - A model for conformational diseases
    • Carrell RW, Lomas DA: Alpha1-antitrypsin deficiency - a model for conformational diseases. N Engl J Med 2002, 346:45-53.
    • (2002) N Engl J Med , vol.346 , pp. 45-53
    • Carrell, R.W.1    Lomas, D.A.2
  • 29
    • 3342889562 scopus 로고    scopus 로고
    • Different conformational changes within the F-helix occur during serpin folding, polymerization, and proteinase inhibition
    • Cabrita LD, Dai W, Bottomley SP: Different conformational changes within the F-helix occur during serpin folding, polymerization, and proteinase inhibition. Biochemistry 2004, 43:9834-9839.
    • (2004) Biochemistry , vol.43 , pp. 9834-9839
    • Cabrita, L.D.1    Dai, W.2    Bottomley, S.P.3
  • 30
    • 1842532017 scopus 로고    scopus 로고
    • Analyses of hepatocellular proliferation in a mouse model of alpha-1-antitrypsin deficiency
    • Rudnick DA, Liao Y, An JK, et al.: 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
  • 31
    • 0037816258 scopus 로고    scopus 로고
    • Elucidation of the molecular logic by which misfolded alpha 1-antitrypsin is preferentially selected for degradation
    • Wu Y, Swulius MT, Moremen KW, Sifers RN: Elucidation of the molecular logic by which misfolded alpha 1-antitrypsin is preferentially selected for degradation. Proc Natl Acad Sci U S A, 2003, 100:8229-8234.
    • (2003) Proc Natl Acad Sci U S A , vol.100 , pp. 8229-8234
    • Wu, Y.1    Swulius, M.T.2    Moremen, K.W.3    Sifers, R.N.4
  • 32
    • 2142768895 scopus 로고    scopus 로고
    • Activation of endoplasmic reticulum-specific stress responses associated with the conformational disease Z alpha 1-antitrypsin deficiency
    • Lawless MW, Creene CM, Mulgrew A, et al.: Activation of endoplasmic reticulum-specific stress responses associated with the conformational disease Z alpha 1-antitrypsin deficiency. J Immunol, 2004, 172:5722-5726.
    • (2004) J Immunol , vol.172 , pp. 5722-5726
    • Lawless, M.W.1    Creene, C.M.2    Mulgrew, A.3
  • 33
    • 0036840471 scopus 로고    scopus 로고
    • Fasting in alpha1-antitrypsin deficient liver: Constitutive [correction of consultative] activation of autophagy
    • Teckman JH, An JK, Loethen S, Perlmutter DH: Fasting in alpha1-antitrypsin deficient liver: constitutive [correction of consultative] activation of autophagy. Am J Physiol Gastrointest Liver Physiol 2002, 283:G1156-GG165.
    • (2002) Am J Physiol Gastrointest Liver Physiol , vol.283
    • Teckman, J.H.1    An, J.K.2    Loethen, S.3    Perlmutter, D.H.4
  • 34
    • 0033963160 scopus 로고    scopus 로고
    • Role of ubiquitin in proteasomal degradation of mutant alpha(1)-antitrypsin Z in the endoplasmic reticulum
    • Teckman JH, Gilmore R, Perlmutter DH: Role of ubiquitin in proteasomal degradation of mutant alpha(1)-antitrypsin Z in the endoplasmic reticulum. Am J Physiol Gastrointest Liver Physiol 2000, 278:G39-G48.
    • (2000) Am J Physiol Gastrointest Liver Physiol , vol.278
    • Teckman, J.H.1    Gilmore, R.2    Perlmutter, D.H.3
  • 35
    • 15844416550 scopus 로고    scopus 로고
    • The endoplasmic reticulum degradation pathway for mutant secretory proteins alpha1-antitrypsin Z and S is distinct from that for an unassembled membrane protein
    • Teckman JH, Perlmutter DH: The endoplasmic reticulum degradation pathway for mutant secretory proteins alpha1-antitrypsin Z and S is distinct from that for an unassembled membrane protein. J Biol Chem 1996, 271:13215-13220.
    • (1996) J Biol Chem , vol.271 , pp. 13215-13220
    • Teckman, J.H.1    Perlmutter, D.H.2
  • 36
    • 0034695450 scopus 로고    scopus 로고
    • Glucosidase and mannosidase inhibitors mediate increased secretion of mutant alpha1 antitrypsin Z
    • Marcus NY, Perlmutter DH: Glucosidase and mannosidase inhibitors mediate increased secretion of mutant alpha1 antitrypsin Z. J Biol Chem 2000, 275:1987-1992.
    • (2000) J Biol Chem , vol.275 , pp. 1987-1992
    • Marcus, N.Y.1    Perlmutter, D.H.2
  • 37
    • 0027295822 scopus 로고
    • Alpha 1-antitrypsin Siiyama (Ser53-->Phe). Further evidence for intracellular loop-sheet polymerization
    • Lomas DA, Lomas DA, Finch JT, et al.: Alpha 1-antitrypsin Siiyama (Ser53-->Phe). Further evidence for intracellular loop-sheet polymerization. J Biol Chem 1993, 268:15333-15335.
    • (1993) J Biol Chem , vol.268 , pp. 15333-15335
    • Lomas, D.A.1    Lomas, D.A.2    Finch, J.T.3
  • 38
    • 0029012309 scopus 로고
    • Alpha 1-Antitrypsin Mmalton (Phe52-deleted) forms loop-sheet polymers in vivo. Evidence for the C sheet mechanism of polymerization
    • Lomas DA, Elliott PR, Sidhar SK, et al.: Alpha 1-Antitrypsin Mmalton (Phe52-deleted) forms loop-sheet polymers in vivo. Evidence for the C sheet mechanism of polymerization. J Biol Chem 1995, 270:16864-16870.
    • (1995) J Biol Chem , vol.270 , pp. 16864-16870
    • Lomas, D.A.1    Elliott, P.R.2    Sidhar, S.K.3
  • 39
    • 0037104966 scopus 로고    scopus 로고
    • Rapid screening for alpha1-antitrypsin deficiency in patients with chronic obstructive pulmonary disease using dried blood specimens
    • Rodriguez F, Jardi R, Costa X, et al.: Rapid screening for alpha1-antitrypsin deficiency in patients with chronic obstructive pulmonary disease using dried blood specimens. Am J Respir Crit Care Med 2002, 166:814-817.
    • (2002) Am J Respir Crit Care Med , vol.166 , pp. 814-817
    • Rodriguez, F.1    Jardi, R.2    Costa, X.3
  • 40
    • 0036341010 scopus 로고    scopus 로고
    • Respiratory symptoms and lung function in young adults with severe alpha(1)-antitrypsin deficiency (PiZZ)
    • Piitulainen E, Sveger T: Respiratory symptoms and lung function in young adults with severe alpha(1)-antitrypsin deficiency (PiZZ). Thorax 2002, 57:705-708.
    • (2002) Thorax , vol.57 , pp. 705-708
    • Piitulainen, E.1    Sveger, T.2
  • 41
    • 0031035138 scopus 로고    scopus 로고
    • Young adults with alpha 1-antitrypsin deficiency identified neonatally: Their health, knowledge about and adaptation to the high-risk condition
    • Sveger T, Thelin T, McNeil TF: Young adults with alpha 1-antitrypsin deficiency identified neonatally: their health, knowledge about and adaptation to the high-risk condition. Acta Paediatr 1997, 86:37-40.
    • (1997) Acta Paediatr , vol.86 , pp. 37-40
    • Sveger, T.1    Thelin, T.2    McNeil, T.F.3
  • 42
    • 16544381001 scopus 로고    scopus 로고
    • Lack of effect of oral 4-phenylbutyrate on serum alpha-1-antitrypsin in patients with alpha-1-antitrypsin deficiency: A preliminary study
    • Teckman JH: Lack of effect of oral 4-phenylbutyrate on serum alpha-1-antitrypsin in patients with alpha-1-antitrypsin deficiency: a preliminary study. J Pediatr Gastroenterol Nutr 2004, 39:34-37.
    • (2004) J Pediatr Gastroenterol Nutr , vol.39 , pp. 34-37
    • Teckman, J.H.1
  • 43
    • 5144228167 scopus 로고    scopus 로고
    • Gene therapy for human alphal-antitrypsin deficiency in an animal model using SV40-derived vectors
    • Duan YY, Wu J, Zhu JL, et al.: Gene therapy for human alphal-antitrypsin deficiency in an animal model using SV40-derived vectors. Gastroenterology 2004, 127:1222-1232.
    • (2004) Gastroenterology , vol.127 , pp. 1222-1232
    • Duan, Y.Y.1    Wu, J.2    Zhu, J.L.3
  • 44
    • 0030999090 scopus 로고    scopus 로고
    • Heterozygous alpha 1-antitrypsin phenotypes in patients with end stage liver disease
    • Eigenbrodt ML, McCashland TM, Dy RM, et al.: Heterozygous alpha 1-antitrypsin phenotypes in patients with end stage liver disease. Am J Gastroenterol 1997, 92:602-607.
    • (1997) Am J Gastroenterol , vol.92 , pp. 602-607
    • Eigenbrodt, M.L.1    McCashland, T.M.2    Dy, R.M.3
  • 45
    • 0033652835 scopus 로고    scopus 로고
    • Chronic liver disease in heterozygous alpha1-antitrypsin deficiency PiZ
    • Fischer HP, Ortiz-Pallardo ME, Ko Y, et al.: Chronic liver disease in heterozygous alpha1-antitrypsin deficiency PiZ. J Hepatol 2000, 33:883-892.
    • (2000) J Hepatol , vol.33 , pp. 883-892
    • Fischer, H.P.1    Ortiz-Pallardo, M.E.2    Ko, Y.3
  • 46
    • 0026065089 scopus 로고
    • Liver disease and heterozygous alpha1-antitrypsin deficiency
    • Pittschieler K: Liver disease and heterozygous alpha1-antitrypsin deficiency. Acta Paediatr Scand 1991, 80:323-327.
    • (1991) Acta Paediatr Scand , vol.80 , pp. 323-327
    • Pittschieler, K.1
  • 47
    • 0027220733 scopus 로고
    • Association between heterozygous alpha 1-antitrypsin deficiency and genetic hemochromatosis
    • Kaserbacher R, Propst T, Propst A, et al.: Association between heterozygous alpha 1-antitrypsin deficiency and genetic hemochromatosis. Hepatology 1993, 18:707-708.
    • (1993) Hepatology , vol.18 , pp. 707-708
    • Kaserbacher, R.1    Propst, T.2    Propst, A.3
  • 48
    • 0026737470 scopus 로고
    • High prevalence of viral infection in adults with homozygous and heterozygous alpha 1-antitrypsin deficiency and chronic liver disease
    • Propst T, Propst A, Dietze O, et al.: High prevalence of viral infection in adults with homozygous and heterozygous alpha 1-antitrypsin deficiency and chronic liver disease. Ann Intern Med 1992, 117:641-645.
    • (1992) Ann Intern Med , vol.117 , pp. 641-645
    • Propst, T.1    Propst, A.2    Dietze, O.3
  • 49
    • 0019449013 scopus 로고
    • Heterozygous MZ alpha 1-antitrypsin deficiency in adults with chronic active hepatitis and cryptogenic cirrhosis
    • Hodges JR, Millward-Sadler GH, Barbatis C, Wright R: Heterozygous MZ alpha 1-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
  • 50
    • 0034659888 scopus 로고    scopus 로고
    • Is heterozygous alpha-1-antitrypsin deficiency type PIZ a risk factor for primary liver carcinoma?
    • Zhou H, Ortiz-Pallardo ME, Ko Y, Fischer HP: Is heterozygous alpha-1-antitrypsin deficiency type PIZ a risk factor for primary liver carcinoma? Cancer 2000, 88:2668-2676.
    • (2000) Cancer , vol.88 , pp. 2668-2676
    • Zhou, H.1    Ortiz-Pallardo, M.E.2    Ko, Y.3    Fischer, H.P.4
  • 51
    • 0032173068 scopus 로고    scopus 로고
    • Enhanced phenotypic expression of alpha-1-antitrypsin deficiency in an MZ heterozygote with chronic hepatitis C
    • Banner BF, Karamitsios N, Smith L, Bonkovsky HL: Enhanced phenotypic expression of alpha-1-antitrypsin deficiency in an MZ heterozygote with chronic hepatitis C. Am J Gastroenterol 1998, 93:1541-1545.
    • (1998) Am J Gastroenterol , vol.93 , pp. 1541-1545
    • Banner, B.F.1    Karamitsios, N.2    Smith, L.3    Bonkovsky, H.L.4
  • 52
    • 0032900570 scopus 로고    scopus 로고
    • Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis
    • Mahadeva K, Chang WS, Dafforn TR, et al.: Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis. J Clin Invest 1999, 103:999-1006.
    • (1999) J Clin Invest , vol.103 , pp. 999-1006
    • Mahadeva, K.1    Chang, W.S.2    Dafforn, T.R.3


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