[Alpha-1 antitrypsin deficiency 50 years later].

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Article

[Alpha-1 antitrypsin deficiency 50 years later].

Bulletin de l'Academie nationale de medecine - 1 Jan 2000

Mornex Jean-François

Abstract excerpt

Alpha-1 antitrypsin deficiency is a frequent genetic disorder associated with pulmonary emphysema in smokers and with liver cirrhosis. Aside from lung or liver transplantation, only replacement therapy can currently slow the progression of emphysema. Progress in the pathogenesis of this disorder (protein misfolding, RER aggregation) is opening the way to new strategies such as proteostasis control. Alpha-1...

Topics

Alleles
Disease Progression
Genetic Predisposition to Disease
Humans
Liver Cirrhosis
Protein Folding
Pulmonary Emphysema
Serpins
alpha 1-Antitrypsin
alpha 1-Antitrypsin Deficiency

Source

Open in PubMed

Identifiers

PMID: 26753407

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 31 Aug 2026.

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