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The Relationship between Plasma Alpha-1-Antitrypsin Polymers and Lung or Liver Function in ZZ Alpha-1-Antitrypsin-Deficient Patients

Alpha-1-Antitrypsin (AAT) is a protein of the SERPINA1 gene. A single amino acid mutation (Lys342Glu) results in an expression of misfolded Z-AAT protein, which has a high propensity to intra- and extra-cellular polymerization. Here, we asked whether levels of circulating Z-AAT polymers are associat...

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Detalles Bibliográficos
Autores principales: Sark, Annelot D., Fromme, Malin, Olejnicka, Beata, Welte, Tobias, Strnad, Pavel, Janciauskiene, Sabina, Stolk, Jan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8945708/
https://www.ncbi.nlm.nih.gov/pubmed/35327571
http://dx.doi.org/10.3390/biom12030380