SEARCH

SEARCH BY CITATION

References

  • 1
    Silverman EK, Sandhaus RA. Alpha1-antitrypsin deficiency. N Engl J Med 2009; 360: 2749-2757.
  • 2
    Ekeowa UI, Freeke J, Miranda E, Gooptu B, Bush MF, Perez J, et al. Defining the mechanism of polymerization in the serpinopathies. Proc Natl Acad Sci U S A 2010; 107: 17146-17151.
  • 3
    Fairbanks KD, Tavill AS. Liver disease in alpha 1-antitrypsin deficiency: a review. Am J Gastroenterol 2008; 103: 2136-2141.
    Direct Link:
  • 4
    Eriksson S, Propst A, Sueger T, Teckman J. 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.
  • 5
    Chapman KR, Stockley RA, Dawkins C, Wilkes MM, Navickis RJ. Augmentation therapy for alpha1 antitrypsin deficiency: a meta-analysis. COPD 2009; 6: 177-184.
  • 6
    Dirksen A, Piitulainen E, Parr DG, Deng C, Wencker M, Shaker SB, et al. Exploring the role of CT densitometry: a randomised study of augmentation therapy in alpha1-antitrypsin deficiency. Eur Respir J 2009; 33: 1345-1353.
  • 7
    Kemmer N, Kaiser T, Zacharias V, Neff GW. Alpha-1-antitrypsin deficiency: outcomes after liver transplantation. Transplant Proc 2008; 40: 1492-1494.
  • 8
    Gooptu B, Lomas DA. Conformational pathology of the serpins: themes, variations, and therapeutic strategies. Annu Rev Biochem 2009; 78: 147-176.
  • 9
    Mallya M, Phillips RL, Saldanha SA, Gooptu B, Brown SC, Termine DJ, et al. Small molecules block the polymerization of Z alpha1-antitrypsin and increase the clearance of intracellular aggregates. J Med Chem 2007; 50: 5357-5363.
  • 10
    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.
  • 11
    Hidvegi T, Ewing M, Hale P, Dippold C, Beckett C, Kemp C, et al. An autophagy-enhancing drug promotes degradation of mutant alpha 1-antitrypsin Z and reduces hepatic fibrosis. Science 2010; 329: 229-232.
  • 12
    Sifers RN. Clearing conformational disease. Science 2010; 329: 154-155.
  • 13
    Kaushal S, Annamali M, Blomenkamp K, Rudnick D, Halloran D, Brunt EM, et al. Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse model. Exp Biol Med (Maywood) 2010; 235: 700-709.
  • 14
    Sasaki M, Miyakoshi M, Sato Y, Nakanuma Y. Autophagy mediates the process of cellular senescence characterizing bile duct damages in primary biliary cirrhosis. Lab Invest 2010; 90: 835-843.