Literature DB >> 9713452

Genetics and respiratory disease. 2. Alpha 1-antitrypsin deficiency, cirrhosis and emphysema.

R Mahadeva1, D A Lomas.   

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Year:  1998        PMID： 9713452      PMCID： PMC1745245          DOI： 10.1136/thx.53.6.501

Source DB:  PubMed          Journal:  Thorax        ISSN： 0040-6376            Impact factor:   9.139


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  56 in total

1.  Formation and properties of C1-inhibitor polymers.

Authors:  P A Patston; J Hauert; M Michaud; M Schapira
Journal:  FEBS Lett       Date:  1995-07-24       Impact factor: 4.124

2.  Preparation and characterization of latent alpha 1-antitrypsin.

Authors:  D A Lomas; P R Elliott; W S Chang; M R Wardell; R W Carrell
Journal:  J Biol Chem       Date:  1995-03-10       Impact factor: 5.157

3.  Thromboembolic disease due to thermolabile conformational changes of antithrombin Rouen-VI (187 Asn-->Asp)

Authors:  D Bruce; D J Perry; J Y Borg; R W Carrell; M R Wardell
Journal:  J Clin Invest       Date:  1994-12       Impact factor: 14.808

4.  A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency.

Authors:  Y Wu; I Whitman; E Molmenti; K Moore; P Hippenmeyer; D H Perlmutter
Journal:  Proc Natl Acad Sci U S A       Date:  1994-09-13       Impact factor: 11.205

5.  COOH-terminal substitutions in the serpin C1 inhibitor that cause loop overinsertion and subsequent multimerization.

Authors:  E Eldering; E Verpy; D Roem; T Meo; M Tosi
Journal:  J Biol Chem       Date:  1995-02-10       Impact factor: 5.157

6.  A thermostable mutation located at the hydrophobic core of alpha 1-antitrypsin suppresses the folding defect of the Z-type variant.

Authors:  J Kim; K N Lee; G S Yi; M H Yu
Journal:  J Biol Chem       Date:  1995-04-14       Impact factor: 5.157

7.  Mutations which impede loop/sheet polymerization enhance the secretion of human alpha 1-antitrypsin deficiency variants.

Authors:  S K Sidhar; D A Lomas; R W Carrell; R C Foreman
Journal:  J Biol Chem       Date:  1995-04-14       Impact factor: 5.157

8.  The effect of the Z mutation on the ability of alpha 1-antitrypsin to prevent neutrophil mediated tissue damage.

Authors:  C G Llewellyn-Jones; D A Lomas; R W Carrell; R A Stockley
Journal:  Biochim Biophys Acta       Date:  1994-11-29

9.  Antithrombin-TRI (Ala382 to Thr) causing severe thromboembolic tendency undergoes the S-to-R transition and is associated with a plasma-inactive high-molecular-weight complex of aggregated antithrombin.

Authors:  V S Lindo; V V Kakkar; M Learmonth; E Melissari; F Zappacosta; M Panico; H R Morris
Journal:  Br J Haematol       Date:  1995-03       Impact factor: 6.998

10.  alpha 1-Antitrypsin Mmalton (Phe52-deleted) forms loop-sheet polymers in vivo. Evidence for the C sheet mechanism of polymerization.

Authors:  D A Lomas; P R Elliott; S K Sidhar; R C Foreman; J T Finch; D W Cox; J C Whisstock; R W Carrell
Journal:  J Biol Chem       Date:  1995-07-14       Impact factor: 5.157
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  25 in total

1.  Hepatopulmonary syndromes.

Authors:  M J Krowka
Journal:  Gut       Date:  2000-01       Impact factor: 23.059

2.  Inactive conformation of the serpin alpha(1)-antichymotrypsin indicates two-stage insertion of the reactive loop: implications for inhibitory function and conformational disease.

Authors:  B Gooptu; B Hazes; W S Chang; T R Dafforn; R W Carrell; R J Read; D A Lomas
Journal:  Proc Natl Acad Sci U S A       Date:  2000-01-04       Impact factor: 11.205

Review 3.  Genetics and pulmonary medicine. 9. Molecular genetics of chronic obstructive pulmonary disease.

Authors:  P J Barnes
Journal:  Thorax       Date:  1999-03       Impact factor: 9.139

4.  How the serpin α1-proteinase inhibitor folds.

Authors:  Klavs Dolmer; Peter G W Gettins
Journal:  J Biol Chem       Date:  2012-02-13       Impact factor: 5.157

Review 5.  Lung injury and lung cancer caused by cigarette smoke-induced oxidative stress: Molecular mechanisms and therapeutic opportunities involving the ceramide-generating machinery and epidermal growth factor receptor.

Authors:  Tzipora Goldkorn; Simone Filosto; Samuel Chung
Journal:  Antioxid Redox Signal       Date:  2014-07-01       Impact factor: 8.401

Review 6.  Cystic fibrosis modifier genes.

Authors:  Jane Davies; Eric Alton; Uta Griesenbach
Journal:  J R Soc Med       Date:  2005       Impact factor: 5.344

Review 7.  Inhibitory serpins. New insights into their folding, polymerization, regulation and clearance.

Authors:  Peter G W Gettins; Steven T Olson
Journal:  Biochem J       Date:  2016-08-01       Impact factor: 3.857

8.  Respiratory symptoms and lung function in young adults with severe alpha(1)-antitrypsin deficiency (PiZZ).

Authors:  E Piitulainen; T Sveger
Journal:  Thorax       Date:  2002-08       Impact factor: 9.139

9.  Decreased expression of interleukin 13 in human lung emphysema.

Authors:  A Boutten; M Bonay; S Laribe; G Leseche; Y Castier; V Leçon-Malas; M Fournier; G Durand; M Aubier; M Dehoux; B Crestani
Journal:  Thorax       Date:  2004-10       Impact factor: 9.139

Review 10.  alpha1-Antitrypsin deficiency . 6: new and emerging treatments for alpha1-antitrypsin deficiency.

Authors:  R A Sandhaus
Journal:  Thorax       Date:  2004-10       Impact factor: 9.139
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