-
Inactive conformation of the serpin alpha(1)-antichymotrypsin indicates two-stage insertion of the reactive loop: implications for inhibitory function and conformational disease.
Proc Natl Acad Sci U S A. 2000 Jan 4;97(1):67-72
PMID: 10618372
-
Chemotactic activity of elastin-derived peptides.
J Clin Invest. 1980 Oct;66(4):859-62
PMID: 6903189
-
Topography of a 2.0 A structure of alpha1-antitrypsin reveals targets for rational drug design to prevent conformational disease.
Protein Sci. 2000 Jul;9(7):1274-81
PMID: 10933492
-
Evolving concepts in the pathogenesis of chronic obstructive pulmonary disease.
Clin Chest Med. 2000 Dec;21(4):621-32
PMID: 11194774
-
Prevention of polymerization of M and Z alpha1-Antitrypsin (alpha1-AT) with trimethylamine N-oxide. Implications for the treatment of alpha1-at deficiency.
Am J Respir Cell Mol Biol. 2001 Jun;24(6):727-32
PMID: 11415938
-
Dissecting glycoprotein quality control in the secretory pathway.
Trends Biochem Sci. 2001 Oct;26(10):619-24
PMID: 11590015
-
The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes.
J Biol Chem. 2001 Nov 30;276(48):44865-72
PMID: 11577074
-
Alpha1-antitrypsin deficiency--a model for conformational diseases.
N Engl J Med. 2002 Jan 3;346(1):45-53
PMID: 11778003
-
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. 2002 Mar 1;277(9):6771-4
PMID: 11773044
-
Mutant Neuroserpin (S49P) that causes familial encephalopathy with neuroserpin inclusion bodies is a poor proteinase inhibitor and readily forms polymers in vitro.
J Biol Chem. 2002 May 10;277(19):17367-73
PMID: 11880376
-
Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema.
Am J Respir Cell Mol Biol. 2002 Jun;26(6):723-30
PMID: 12034572
-
Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury.
J Clin Invest. 2002 Dec;110(11):1579-83
PMID: 12464659
-
Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis.
J Clin Invest. 2002 Dec;110(11):1591-7
PMID: 12464661
-
alpha 1-antitrypsin deficiency in early childhood.
Pediatrics. 1978 Jul;62(1):22-5
PMID: 308215
-
Lung lavage fluid from patients with alpha 1-proteinase inhibitor deficiency or chronic obstructive bronchitis: anti-elastase function and cell profile.
Clin Sci (Lond). 1987 Mar;72(3):373-81
PMID: 2434283
-
Z-type alpha 1-antitrypsin is less competent than M1-type alpha 1-antitrypsin as an inhibitor of neutrophil elastase.
J Clin Invest. 1987 Nov;80(5):1366-74
PMID: 3500183
-
The inhibitory complex of human alpha 1-proteinase inhibitor and human leukocyte elastase is a neutrophil chemoattractant.
J Exp Med. 1988 May 1;167(5):1608-15
PMID: 3259253
-
Soluble aggregates of the human PiZ alpha 1-antitrypsin variant are degraded within the endoplasmic reticulum by a mechanism sensitive to inhibitors of protein synthesis.
J Biol Chem. 1992 Jan 15;267(2):1072-80
PMID: 1530934
-
The mechanism of Z alpha 1-antitrypsin accumulation in the liver.
Nature. 1992 Jun 18;357(6379):605-7
PMID: 1608473
-
The molecular basis of alpha 1-antichymotrypsin deficiency in a heterozygote with liver and lung disease.
J Hepatol. 1993 Jul;18(3):313-21
PMID: 8228125
-
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 Sep 13;91(19):9014-8
PMID: 8090762
-
Mutations which impede loop/sheet polymerization enhance the secretion of human alpha 1-antitrypsin deficiency variants.
J Biol Chem. 1995 Apr 14;270(15):8393-6
PMID: 7721731
-
What do dysfunctional serpins tell us about molecular mobility and disease?
Nat Struct Biol. 1995 Feb;2(2):96-113
PMID: 7749926
-
The Z type variation of human alpha 1-antitrypsin causes a protein folding defect.
Nat Struct Biol. 1995 May;2(5):363-7
PMID: 7664092
-
Inhibitory conformation of the reactive loop of alpha 1-antitrypsin.
Nat Struct Biol. 1996 Aug;3(8):676-81
PMID: 8756325
-
Structural explanation for the deficiency of S alpha 1-antitrypsin.
Nat Struct Biol. 1996 Nov;3(11):910-1
PMID: 8901864
-
Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema.
Am J Respir Cell Mol Biol. 1998 May;18(5):670-4
PMID: 9569237
-
Inhibition of intracellular degradation increases secretion of a mutant form of alpha1-antitrypsin associated with profound deficiency.
J Clin Invest. 1998 Jun 15;101(12):2693-701
PMID: 9637703
-
A kinetic mechanism for the polymerization of alpha1-antitrypsin.
J Biol Chem. 1999 Apr 2;274(14):9548-55
PMID: 10092640
-
Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis.
J Clin Invest. 1999 Apr;103(7):999-1006
PMID: 10194472
-
Familial dementia caused by polymerization of mutant neuroserpin.
Nature. 1999 Sep 23;401(6751):376-9
PMID: 10517635
-
Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency.
Proc Natl Acad Sci U S A. 2000 Feb 15;97(4):1796-801
PMID: 10677536