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

Lignende værker

• Synthesis of stress proteins is increased in individuals with homozygous PiZZ alpha 1-antitrypsin deficiency and liver disease.
  af: Perlmutter, D H, et al.
  Udgivet: (1989)
• Survival in severe alpha-1-antitrypsin deficiency (PiZZ)
  af: Nilsson Jan-Åke, et al.
  Udgivet: (2010-04-01)
• Survival in severe alpha-1-antitrypsin deficiency (PiZZ)
  af: Tanash, Hanan A, et al.
  Udgivet: (2010)
• Elastase regulates the synthesis of its inhibitor, alpha 1-proteinase inhibitor, and exaggerates the defect in homozygous PiZZ alpha 1 PI deficiency.
  af: Perlmutter, D H, et al.
  Udgivet: (1988)
• Progression and Augmentation Therapy in PiSZ and PiZZ Alpha-1 Antitrypsin Deficiency: A Longitudinal Functional and Densitometric Study
  af: Soha Esmaili, et al.
  Udgivet: (2025-04-01)