Intrahepatic heteropolymerization of M and Z alpha-1-antitrypsin

The α-1-antitrypsin (or alpha-1-antitrypsin, A1AT) Z variant is the primary cause of severe A1AT deficiency and forms polymeric chains that aggregate in the endoplasmic reticulum of hepatocytes. Around 2%–5% of Europeans are heterozygous for the Z and WT M allele, and there is evidence of increased...

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Detalles Bibliográficos
Autores principales: Laffranchi, Mattia, Elliston, Emma L.K., Miranda, Elena, Perez, Juan, Ronzoni, Riccardo, Jagger, Alistair M., Heyer-Chauhan, Nina, Brantly, Mark L., Fra, Annamaria, Lomas, David A., Irving, James A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Clinical Investigation 2020
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7453904/
https://www.ncbi.nlm.nih.gov/pubmed/32699193
http://dx.doi.org/10.1172/jci.insight.135459

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7453904/
https://www.ncbi.nlm.nih.gov/pubmed/32699193
http://dx.doi.org/10.1172/jci.insight.135459

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