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Fibrosis-Related Gene Profiling in Liver Biopsies of PiZZ α1-Antitrypsin Children with Different Clinical Courses

PiZZ (Glu342Lys) α1-antitrypsin deficiency (AATD) is characterized by intrahepatic AAT polymerization and is a risk factor for liver disease development in children. The majority of PiZZ children are disease free, hence this mutation alone is not sufficient to cause the disease. We investigated Z-AA...

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Detalles Bibliográficos
Autores principales: Kamp, Jan C., Kappe, Naomi N., Moro, Carlos Fernández, Fuge, Jan, Kuehnel, Mark P., Wrenger, Sabine, Welte, Tobias, van Hoek, Bart, Jonigk, Danny D., Khedoe, Padmini P. S. J., Strnad, Pavel, Björnstedt, Mikael, Stolk, Jan, Janciauskiene, Sabina, Nemeth, Antal
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9916468/
https://www.ncbi.nlm.nih.gov/pubmed/36768808
http://dx.doi.org/10.3390/ijms24032485