-
Cirrhosis associated with alpha-1-antitrypsin deficiency: a previously unrecognized inherited disorder.
J Lab Clin Med. 1969 Jun;73(6):934-9
PMID: 4182334
-
The alpha 1-antitrypsin gene and its deficiency states.
Trends Genet. 1989 Dec;5(12):411-7
PMID: 2696185
-
Replacement therapy of alpha 1-antitrypsin deficiency. Reversal of protease-antiprotease imbalance within the alveolar structures of PiZ subjects.
J Clin Invest. 1981 Nov;68(5):1158-65
PMID: 7028785
-
Structure and variation of human alpha 1-antitrypsin.
Nature. 1982 Jul 22;298(5872):329-34
PMID: 7045697
-
Human alpha 1-proteinase inhibitor. Crystal structure analysis of two crystal modifications, molecular model and preliminary analysis of the implications for function.
J Mol Biol. 1984 Aug 15;177(3):531-57
PMID: 6332197
-
Complete sequence of the cDNA for human alpha 1-antitrypsin and the gene for the S variant.
Biochemistry. 1984 Oct 9;23(21):4828-37
PMID: 6093867
-
Synthesis in E. coli of alpha 1-antitrypsin variants of therapeutic potential for emphysema and thrombosis.
Nature. 1985 Jan 10-18;313(5998):149-51
PMID: 3880873
-
Elastases and emphysema. Current assessment of the protease-antiprotease hypothesis.
Am Rev Respir Dis. 1985 Aug;132(2):417-33
PMID: 3896082
-
Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency.
N Engl J Med. 1986 Mar 20;314(12):736-9
PMID: 3485248
-
Expression of the alpha-1-antitrypsin gene in mononuclear phagocytes of normal and alpha-1-antitrypsin-deficient individuals.
J Clin Invest. 1986 Jun;77(6):1952-61
PMID: 3486887
-
Identification of a second mutation in the protein-coding sequence of the Z type alpha 1-antitrypsin gene.
J Biol Chem. 1986 Dec 5;261(34):15989-94
PMID: 3491072
-
alpha 1-Antitrypsin: molecular pathology, leukocytes, and tissue damage.
J Clin Invest. 1986 Dec;78(6):1427-31
PMID: 3537008
-
Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema.
N Engl J Med. 1987 Apr 23;316(17):1055-62
PMID: 3494198
-
Clonal gene therapy: transplanted mouse fibroblast clones express human alpha 1-antitrypsin gene in vivo.
Science. 1987 Aug 14;237(4816):762-4
PMID: 3497452
-
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
-
Molecular basis of alpha-1-antitrypsin deficiency.
Am J Med. 1988 Jun 24;84(6A):13-31
PMID: 3289385
-
Structure, function, and control of neutrophil proteinases.
Am J Med. 1988 Jun 24;84(6A):37-42
PMID: 2454577
-
Alpha-1-antitrypsin augmentation therapy for alpha-1-antitrypsin deficiency.
Am J Med. 1988 Jun 24;84(6A):52-62
PMID: 3289387
-
Biochemical efficacy and safety of monthly augmentation therapy for alpha 1-antitrypsin deficiency.
JAMA. 1988 Sep 2;260(9):1259-64
PMID: 3261353
-
Repair of the secretion defect in the Z form of alpha 1-antitrypsin by addition of a second mutation.
Science. 1988 Dec 23;242(4886):1700-2
PMID: 2904702
-
The alpha 1-antitrypsin gene and its mutations. Clinical consequences and strategies for therapy.
Chest. 1989 Jan;95(1):196-208
PMID: 2642408
-
Lung function, smoking and survival in severe alpha 1-antitrypsin deficiency, PiZZ.
J Clin Epidemiol. 1988;41(12):1157-65
PMID: 3264848
-
Disruption of the 290-342 salt bridge is not responsible for the secretory defect of the PiZ alpha 1-antitrypsin variant.
J Biol Chem. 1989 Feb 15;264(5):2997-3001
PMID: 2563374
-
Intracellular traffic of newly synthesized proteins. Current understanding and future prospects.
J Clin Invest. 1989 Mar;83(3):739-51
PMID: 2646317
-
Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice.
J Clin Invest. 1989 Apr;83(4):1183-90
PMID: 2784798
-
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. 1989 Jun 25;264(18):10477-86
PMID: 2567291
-
Interferon beta 2/interleukin 6 modulates synthesis of alpha 1-antitrypsin in human mononuclear phagocytes and in human hepatoma cells.
J Clin Invest. 1989 Jul;84(1):138-44
PMID: 2472425
-
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. 1989 Aug 1;111(3):206-12
PMID: 2787611
-
Rapid, nonradioactive detection of mutations in the human genome by allele-specific amplification.
J Lab Clin Med. 1989 Aug;114(2):105-13
PMID: 2787825
-
The molecular pathology of the serpins.
Mol Biol Med. 1989 Feb;6(1):35-42
PMID: 2666817
-
Molecular basis of the liver and lung disease associated with the alpha 1-antitrypsin deficiency allele Mmalton.
J Biol Chem. 1989 Aug 15;264(23):13938-45
PMID: 2788166
-
Pulmonary deposition and clearance of aerosolized alpha-1-proteinase inhibitor administered to dogs and to sheep.
J Clin Invest. 1989 Oct;84(4):1145-54
PMID: 2794051
-
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. 1989 Oct;84(4):1349-54
PMID: 2794066
-
Synthesis of stress proteins is increased in individuals with homozygous PiZZ alpha 1-antitrypsin deficiency and liver disease.
J Clin Invest. 1989 Nov;84(5):1555-61
PMID: 2553776
-
Fine mapping of chromosome 22 breakpoints within the breakpoint cluster region (bcr) implies a role for bcr exon 3 in determining disease duration in chronic myeloid leukemia.
Am J Hum Genet. 1989 Nov;45(5):729-38
PMID: 2683759
-
Molecular basis of alpha 1-antitrypsin deficiency and emphysema associated with the alpha 1-antitrypsin Mmineral springs allele.
Mol Cell Biol. 1990 Jan;10(1):47-56
PMID: 1967187
-
Antielastases of the human alveolar structures. Implications for the protease-antiprotease theory of emphysema.
J Clin Invest. 1981 Oct;68(4):889-98
PMID: 6169740